Hematology RSS : Gourt

Thriving Multispecialty Group in the Desert Southwest is Seeking 2 Oncologists! :: Utah :: CompHealth Inc

Job 915532 Join a small multispecialty group in the scenic southwest Participate in basic and or clinical research Group focuses on technology and excellence Members of the group come from the best

Well established group looking for a Hematology / Medical Oncologist in SC. :: South Carolina :: CompHealth Inc

Job 919057 Growing practice is seeking an Oncologist to join their growing group. Excellent compensation starting at 325K Production bonus Possible Partnership Light Call 1:3 State of the Art Facility

New Oncology Opportunity - Top Single Specialty Group - South Dakota :: South Dakota :: CompHealth Inc

Job 919235 Opportunity to join the strongest Oncology group in this part of the state. The group is the only Oncology practice in the area and has a captive patient base. Group is located in the Cancer

Medical Oncology Need ? Academic / Clinical ? North Carolina :: North Carolina :: CompHealth Inc

Job 919963 Join a top rated University in an outreach clinic where you will have no call. BC/BE in Medical Oncology Solo clinical practice where your evenings and weekends will be free ? Fellows take

Hematology / Medical Oncology position in the Boston area. :: Massachusetts :: CompHealth Inc

Job 919731 Well established group is seeking a partner for their growing Cancer Center Competitive Salary Bonus Incentives Partnership Offered Strong group that is financially stable Light Call Schedule

Medical Oncology need near Rock and Roll Hall of Fame :: Ohio :: CompHealth Inc

Job 919843 Excellent Long-term earning potential Physician-owned practice Partnership track Call 1:5 weekends Latest technologies and treatments University nearby Outstanding compensation Full benefits

Medical Oncology opening in Pacific Northwest Metro :: Washington :: CompHealth Inc

Job 919932 Seeking a Medical oncologist to join a private practice in a family friendly community. Awesome growth opportunity with partnership. Enjoy the Pacific Northwest Metro area. Competitive Compensation

Medical Oncology Need ? Academic ? North Carolina :: North Carolina :: CompHealth Inc

Job 9110056 Join a top rated University where your expertise in clinical and translational research in GU/GI cancers will be appreciated and rewarded. BC/BE in Medical Oncology Seek physician with expertise

Hematology / Medical Oncology Opportunity in PA :: Pennsylvania :: CompHealth Inc

Job 919326 Private Practice with academic affiliation Competitive Compensation Bonus Structure/ Production Bonus Retention Bonus Sign On Bonus Light Call Schedule Partnership Track Clinical Trials Full

Private Practice Opportunity in South Carolina :: South Carolina :: CompHealth Inc

Job 919804 Join 5 Oncologists in a private group Clinical Trials 1:6 call Practice is open to practicing physicians and fellows Close to the coast Great cost of living Private practice with partnership

Hematology Oncology opportunity in Tennessee! :: Tennessee :: CompHealth Inc

Job 919226 Starting salary range is $275K-$375K Production Bonuses Sign on Bonus Relocation assistance Light Call Retirement 401k Benefits Large service area Strong group Close to a Memphis 4 and 1/2

Private Practice Opportunity in North Dakota :: North Dakota :: CompHealth Inc

Job 9110043 Join a group of 3 Oncologists Opening is due to growth Admit to 1 hospital Great area for the outdoor enthusiast Very good education system Competitive first year salary Partnership potential

Hematology Oncology Need in Chicago, IL :: Illinois :: CompHealth Inc

Job 919942 Solid, established Oncology practice in Chicago seeks to add a Hematologic Oncologist to their team of two. This practice is located in Chicago - not a distant suburb. Ideal candidate be bilingual

Huge salary potential for Oncologist :: Ohio :: CompHealth Inc

Job 9110003 Hospital is seeking 2 Oncologists Hospital wants to have Oncologist in place by August 1 Program has roughly 30 patients per day Work out of 1 office Radiation Oncology on site H1 visa candidates

Hot Oncology Opportunity in Arizona :: Arizona :: CompHealth Inc

Job 919974 Solo Practice Position open due to Community demand Solid referral base Practice Management Services Available Many outdoor recreational activities-outstanding golf, boating, fishing Ideal

Metro Texas Oncology Opportunity :: Texas :: CompHealth Inc

Job 919518 Join a group of three Medical Oncologist's Position is open because of growth Well established group Call is 1:4 10-15 patients per day to start Great schools Competitive salary to start

Medical Director needed in Ohio :: Ohio :: CompHealth Inc

Job 9110004 Hospital is seeking 2 Oncologists - One will become Medical Director Hospital wants to have Oncologist in place by August 1 Program has roughly 30 patients per day Work out of 1 office Radiation

Big Salary Position in Oklahoma :: Oklahoma :: CompHealth Inc

Job 919935 Hospital is bringing on 2 Oncologists Practice out of 1 office Office is 2 blocks from the hospital Large Primary Care group will give referrals Client will consider a H1 visa candidate with

Hem/Onc Opportunity in Florida :: Florida :: CompHealth Inc

Job 9110073 $225 - $250 Salary first year. Salary plus incentives year 2 2 year Partnership track 3 weeks vacation, 1 week CME Call of 1:4 25 years in business Large Metro City Strong support staff

Beautiful Spokane :: Washington :: CompHealth Inc

Job 9110109 Strong Group Needing a new physician due to growth Strong Compensation with Production Bonuses Full Benefits 4 1/2 work week Light Call 1:6 3 weeks starting vacation Looking for a BC/BE

Silibinin can induce differentiation as well as enhance vitamin D3-induced differentiation of human AML cells ex vivo and regulates the levels of differentiation-related transcription factors

Induction of terminal differentiation is a conceptually attractive approach for the therapy of neoplastic diseases. Although vitamin D derivatives (deltanoids) can induce differentiation of AML cells in vitro, so far deltanoids have not been successfully brought to the clinic, due to the likelihood of life-threatening hypercalcemia. Here, we incubated freshly obtained blood cells from patients with AML with a plant antioxidant (PAOx), silibinin (SIL), alone or together with a deltanoid. Twenty patients with AML (all subtypes except M3) were available for this study, and in 14 (70%), SIL (60 µM) either induced differentiation ex vivo, or enhanced differentiation induced by deltanoids, or both. Interestingly, SIL acting alone induced differentiation only in cases in which chromosome aberrations could not be detected. In eleven samples sufficient material was available for a limited analysis of the underlying events. Quantitative RT-PCR showed that differentiation markers were upregulated at the mRNA level by both SIL and deltanoids, suggesting that intracellular signaling pathways upstream of transcription factors (TFs) were activated by these agents. Western analysis for proteins which function as TFs in deltanoid-induced monocytic differentiation, such as members of Jun and C/EBP families, surprisingly demonstrated that SIL upregulated all these TFs in the cases tested. This suggests that although the presence of SIL may not always be sufficient to induce differentiation, it can serve as a differentiation enabling factor for blasts obtained from a large proportion of patients with AML. Thus, SIL/deltanoid combinations warrant further consideration as preventive/therapeutic regimens in human leukaemia. Copyright © 2009 John Wiley & Sons, Ltd.

Burkitt lymphoma versus diffuse large B-cell lymphoma: a practical approach

Burkitt Lymphoma (BL) is listed in the World Health Organization (WHO) classification of lymphoid tumours as an 'aggressive B-cell non-Hodgkin's lymphoma', characterized by a high degree of proliferation of the malignant cells and deregulation of the c-MYC gene. The main diagnostic challenge in BL is to distinguish it from diffuse large B-cell lymphoma (DLBCL). While in children BL and DLBCL types probably do not differ clinically, and the differential diagnosis between BL and DLBCL may theoretically appear clear-cut, in adults daily practice shows the existence of cases that have morphological features, immunophenotypic and cytogenetics intermediate between DLBCL and BL, and cannot be classified with certainty in these categories. Distinguishing between BL and DLBCL is critical, as the two diseases require different management. This review summarizes the current practical approach, including the use of a large panel of antibodies, and cytogenetic and molecular diagnostic techniques, to distinguish between BL, DLBCL and the provisional category of 'B-cell lymphoma, unclassifiable, with features intermediate between diffuse large B-cell lymphoma and Burkitt lymphoma', now listed in the updated WHO classification. Copyright © 2009 John Wiley & Sons, Ltd.

Outcomes for lymphoid malignancies in the Nurses' Health Study (NHS) as compared to the Surveillance, Epidemiology and End Results (SEER) Program

Vital statistics for the lymphoid malignancies obtained from the Surveillance, Epidemiology and End Results (SEER) Program have seldom been directly compared to data from alternative national databases. While SEER is recognized as the standard, some lymphoid malignancies - especially the chronic ones - may be underreported. We compared the incidence, all-cause and cause-specific mortality for Hodgkin's lymphoma (HL), non-Hodgkin's lymphoma (NHL), multiple myeloma (MM) and chronic lymphocytic leukaemia (CLL) in SEER to that in the Nurses' Health Study (NHS), a national cohort study of 121 700 female registered nurses, matching for age and race. In over 2.5 million person-years, the incidence of HL was the same as in SEER (SIR = 1.01 [0.75, 1.26]), while the incidence of NHL, CLL and MM were slightly higher. All-cause mortality was lower for the lymphoid malignancies except for MM, which was the same; there were no differences in cause-specific mortality, except for MM (HR = 1.26 [1.07, 1.48]). Our analysis suggests that, at least among white women, SEER is a reliable data source with respect to lymphoid malignancies. Copyright © 2009 John Wiley & Sons, Ltd.

Dynamic change of T315I BCR-ABL kinase domain mutation in Korean chronic myeloid leukaemia patients during treatment with Abl tyrosine kinase inhibitors

We analysed the dynamic change of imatinib-resistant mutations in BCR-ABL kinase domain focusing on T315I mutation during dasatinib or nilotinib therapy. Fifty-five imatinib-resistant chronic myeloid leukaemia patients (32 patients with imatinib-resistant mutations and 23 patients without mutation) in different disease phases were treated with dasatinib (median 17.3 months) or nilotinib (median 6.8 months). Among the 32 patients with baseline mutation, mutations including M244V, G250E, E255K, M351T, H396R, S417Y, E450K and E459K disappeared in 8 patients and new mutations were detected in 9 patients, all of which were T315I. Among the 23 patients without baseline mutation, 4 patients showed newly developed mutations including T315I, T315I + E459K, M244V and F359V. The T315I was the most frequently detected mutation in imatinib therapy (16%, 9 of 55) as well as in dasatinib or nilotinib therapy (24%, 11 of 44). Patients with imatinib resistant baseline mutations had a higher rate of mutation development during dasatinib or nilotinib treatment compared to patients without baseline mutations (28% vs. 17%). Copyright © 2009 John Wiley & Sons, Ltd.

Clofarabine in the treatment of poor risk acute myeloid leukaemia

Clofarabine is a second generation nucleoside analogue. It inhibits DNA repair and activates the mitochondrial apoptotic pathway leading to cell death. In vitro clofarabine has demonstrated synergy with daunorubicin and Ara-C and in phase II clinical trials has shown promising activity in poor risk Acute myeloid leukaemia (AML) patients. In our institution over a 24 month period 22 AML patients (11 M, 11 F) with poor risk features, deemed unsuitable for standard therapy, were treated with clofarabine, alone (eight patients) or in combination (14 patients) for up to three cycles of treatment. The median age was 67.5 years (24-76) with 16 patients > 60 years. At the time of treatment 18 patients had active AML. Four patients intolerant of standard induction received clofarabine as consolidation. The overall response rate (ORR) for the 18 patients with active AML was 61%, nine patients (50%) achieving a complete response (CR). Induction and consolidation were well tolerated with no unexpected toxicities. Predictably, all patients developed grade 4 neutropenia but the median duration was only 20 days (17-120). Induction mortality was acceptable at 17%. In conclusion, clofarabine (alone or in combination) is active in poor risk AML with an acceptable safety profile and should be considered a potential option in poor risk AML patients. Copyright © 2009 John Wiley & Sons, Ltd.

Clinical characteristics of patients with chronic eosinophilic leukaemia (CEL) harbouring FIP1L1-PDGFRA fusion transcript - results of Polish multicentre study

A small subgroup of patients with hypereosinophilic syndrome (HES) demonstrates imatinib-sensitive fusion transcript - the FIP1L1-PDGFRA (F/P+). These cases are currently diagnosed as chronic eosinophilic leukaemia (CEL). In this paper, we screened 77 patients to estimate the frequency of FIP1L1-PDGFRA transcript among patients with unexplained, long-term hypereosinophilia exceeding 1.5 × 109/L and to analyse the clinical and serological features in F/P+ CEL population. The FIP1L1-PDGFRA chimeric protein was detectable in 16 (14 males and 2 females) out of 77 examined HES patients (20%) by RT-PCR. Two patients suffered from cough at diagnosis. Three out of 16 (18%) patients had no organ involvements, in 5-one organ was affected and in the remaining eight cases - at least two. Eosinophilic organ damage/dysfunction identified splenomegaly in the majority of studied patients. We compared clinical and serological features between CEL F/P+ (n = 16) and HES (n = 61) patients. F/P+ cases had significantly increased WBC and absolute eosinophil count (AEC) at diagnosis (p = 0.008 and 0.02), whereas platelet count was decreased in this population (p = 0.03). Serum B12 and tryptase levels were increased (p = 0.002 and 0.004) in CEL F/P+ patients when compared to HES cases whereas serum IL-5 levels were significantly increased in the latter group (p = 0.01). Male gender and splenomegaly occurred more frequent in CEL F/P+ population (p = 0.002 and 0.0007, respectively). Additionally, patients with F/P+ CEL (n = 16) were compared with F/P- CEL (n = 8). The latter group, was significantly older, had lower AEC and higher platelet count. In conclusion, significant clinical symptoms are infrequent present and splenomegaly remains the most common organ involvement in patients with CEL expressing F/P fusion transcript. Our study confirmed the long-term remission on imatinib in this patient population. Copyright © 2009 John Wiley & Sons, Ltd.

Cytogenetic and molecular responses in chronic phase chronic myeloid leukaemia patients receiving low dose of imatinib for intolerance to standard dose

Imatinib mesylate is the gold standard treatment of chronic myeloid leukaemia (CML) and 400 mg/day is considered the standard dose. Although it is generally well tolerated, some patients require temporary drug discontinuation and permanent dose reduction because of haematological or non-haematological toxicities. Whether or not reduced doses are effective as the standard dose in inducing and/or maintaining complete cytogenetic and molecular response is not clear. We report the outcome of 45 CML patients in early (17) and late (28) chronic phase (CP) in whom, within a series of 250 patients treated with imatinib, reduced the dose of the drug after experiencing adverse events. Median time interval between the start of therapy and dose reduction was 58 days, whereas median administered dose was 300 mg/day. At 6 months from reduction, major cytogenetic responses (MCRs) were observed in 67% of patients, with 58% being complete cytogenetic remission (CCR), and complete molecular response (CMR) were obtained in 18% of patients. At 12 months, all patients who had obtained MCR reached CCR: this was significantly higher in low risk patients (87%) versus non-low risk patients (66 and 46%), and in early phase (82%) versus late phase (53.5%). CMR and major molecular response (MMR) were detected in 20 and 22% of patients, respectively. Low dose imatinib appears effective in patients with intolerance to standard dose, even though long-term effects remain to be established. Copyright © 2009 John Wiley & Sons, Ltd.

Geographic variation and environmental conditions as cofactors in Chlamydia psittaci association with ocular adnexal lymphomas: a comparison between Italian and African samples

A particular extra-nodal lymphoma type arises from B cells of the marginal zone (MZ) of mucosa-associated lymphoid tissue (MALT). The aetiology of MZ lymphomas suggests that they are associated with chronic antigenic stimulation by microbial pathogens, among which Helicobacter pylori-associated gastric MALT lymphoma is the best studied. Recently, MALT lymphomas have been described in the context of chronic conjunctivitis, which can be associated with Chlamydia spp. infection. Studies from Italy showed the presence of Chlamydia psittaci in 87% of ocular adnexal lymphomas (OAL), and C. psittaci has been described in a large part of samples from Austria and Korea as well. However, this finding was not always confirmed by other studies, suggesting that the association with C. psittaci may depend on geographic heterogeneity. Interestingly, none of the studies up to now has been carried out in the African population, where a strong association between infectious agents and the occurrence of human neoplasms has been reported. This study was designed to investigate the possible association of Chlamydia psittaci in cases retrieved from Kenya, compared to cases from Italy. Our results showed that there was a marked variation between the two geographical areas in terms of association with C. psittaci, as 17% (5/30) of the samples from Italy were positive for C. psittaci, whereas no association with this pathogen was observed in any of the African samples (0/9), suggesting that other cofactors may determine the OAL occurrence in those areas. OAL cases are often characterized by down-regulation of p16/INK4a expression and promoter hypermethylation of the p16/INK4a gene. Our results showed a partial methylation of p16/INK4a promoter in C. psittaci-negative cases, whereas no hypermethylation of this gene was found in C. psittaci-positive cases, suggesting that mechanisms other than promoter hypermethylation lead to p16/INK4a silencing in C. psittaci-positive cases. We may conclude that the role of epidemiologic, environmental and genetic factors, must be considered in the aetiology of this disease. Copyright © 2009 John Wiley & Sons, Ltd.

CT-guided percutaneous lung biopsies in patients with haematologic malignancies and undiagnosed pulmonary lesions

We searched the electronic patient database at The University of Texas M. D. Anderson Cancer Center for patients who underwent computed tomography (CT)-guided needle biopsy between January 2001 and December 2005. Inclusion criteria were a known history of haematologic malignancy and a newly detected, undiagnosed pulmonary lesion on chest CT that required tissue sampling for diagnosis; 213 met these criteria. We analysed the biopsy results for diagnostic yield, factors affecting diagnostic yield and effect on treatment. Of 213 procedures, 191 (89.7%) yielded sufficient material for pathologic analysis; 130 (60%) yielded specific diagnoses, while 61 (28.6%) yielded nonspecific benign diagnoses. Lesions larger than 1 cm, cavitary lesions and lung masses were more likely to yield a specific diagnosis than were lesions smaller than 1 cm, lung nodules and consolidations. The most common specific diagnoses were malignancy (62.8%) and infection (34.3%). The latter was more common in patients with leukaemia, cavitary lung lesions or consolidations, active underlying malignancy, neutropenia, respiratory signs and symptoms and/or fever, bone marrow transplant recipients, and in patients receiving chemotherapy. Lung lesions discovered upon follow-up imaging in patients who did not have any respiratory signs/symptoms or fever were mostly malignant. Therapeutic changes were more likely after a specific diagnosis than after a nonspecific diagnosis or a nondiagnostic biopsy (88.4% vs. 18.1%; p < 0.0001). CT-guided lung biopsy has a high diagnostic yield in patients with haematologic malignancies that present with unexplained pulmonary lesions and provides a specific diagnosis in a majority of these patients, leading to therapeutic changes. Copyright © 2009 John Wiley & Sons, Ltd.

Severe pulmonary complications after bortezomib treatment in multiple myeloma

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The root of many evils: indolent large granular lymphocyte leukaemia and associated disorders

Large granular lymphocytes (LGL) leukaemia can arise from either natural killer (NK) cells or cytotoxic T lymphocytes (CTL). The T-cell form of LGL leukaemia has significant overlap with other haematological disorders and autoimmune diseases. Here we provide an overview of LGL biology. We also focus discussion on the indolent LGL leukaemia related disorders and their causal relationships. We then discuss the potential relationships and distinctions between indolent LGL leukaemia and non-malignant clonal lymphocyte expansion that occur in otherwise healthy individuals, especially elder people. Copyright © 2009 John Wiley & Sons, Ltd.

Remission induction, consolidation and novel agents in development for adults with acute myeloid leukaemia

Chemotherapy regimens used for remission induction in AML have not changed significantly over the last several decades. However the recognition of the prognostic value of cytogenetics and genomics has been a major advance which is helping clarify the most optimal post-remission consolidation strategy among various risk groups. We are not only beginning to realize the pitfalls of a 'one-fits-all' approach with intensive, cytarabine-based chemotherapy as the mainstay, but we are finally beginning to reap the rewards of decades of basic, translational, and clinical research. Developing individualized, 'targeted' therapy for each AML patient based on unique molecular features of disease remains a daunting goal yet one that we can now begin to envision. Hypothesis-based study designs - from pre-clinical/laboratory experiments to phase-I and subsequent efficacy trials - provide the foundation for advances in the diagnosis, risk stratification, and treatment for patients with AML. Here we critically review the literature for the management of AML, try to give recommendations regarding the appropriate induction and remission strategy, clarify the role of stem cell transplantation and discuss novel agents on the horizon. Copyright © 2009 John Wiley & Sons, Ltd.

18F-FDG uptake and its clinical relevance in primary gastric lymphoma

We studied the clinical relevance of 18F-fluorodeoxyglucose (18F-FDG) uptake in patients with primary gastric lymphoma underwent positron emission tomography (PET)/ computed tomography (CT) scan. Forty-two patients with primary gastric lymphoma were analysed: 32 diffuse large B-cell lymphomas (DLBCL) and 10 extranodal marginal zone B-cell lymphomas of mucosa-associated lymphoid tissue (MALT lymphomas). The PET/CT scans were compared with clinical and pathologic features, and the results of CT and endoscopy. Nine patients were up-staged based on the results of their PET/CT scan compared to CT (seven DLBCLs, two MALT lymphomas) while six patients were down-staged by the PET/CT scan. The standard uptake value (SUV) was used as an indicator of a lesion with a high metabolic rate. The high SUVmax group, defined as an SUVmax [ge] median value, was significantly associated with an advanced Lugano stage (p < 0.001). Three patients with DLBCL, who showed an initially high SUVmax, died of disease progression. Among 24 patients for whom follow-up PET/CT scan with endoscopy was performed, 11 patients with ulcerative or mucosal lesions showed residual 18F-FDG uptake. All of these gastric lesions were grossly and pathologically benign lesions without evidence of lymphoma cells. In conclusion, PET/CT scan can be used in staging patients with primary gastric lymphoma; however, the residual 18F-FDG uptake observed during follow-up should be interpreted cautiously and should be combined with endoscopy and multiple biopsies of the stomach. Copyright © 2009 John Wiley & Sons, Ltd.

Two novel NPM1 mutations in a therapy-responder AML patient

Nucleophosmin 1 (NPM1) is an abundant phosphoprotein mainly located in the nucleolus but also shuttling between the nucleus and cytoplasm. NPM1 has been proposed to be involved in synthesis and processing of ribosomal RNA, regulation of chromatin structure and transport of rRNA and ribosomal proteins. NPM1 gene is considered to be implicated in human cancer as it is a frequent target of genetic alterations, primarily in haematopoietic neoplasms. We describe a case of a therapy-responder acute myeloid leukaemia (AML) patient bearing two novel NPM1 mutations. Cells' transfection studies indicate that the presence of one of these mutations is associated to an abnormal nucleolar structure, suggesting that NPM1 may contribute to the control of nucleolar integrity. Copyright © 2009 John Wiley & Sons, Ltd.

Experimental and clinical characteristics in myelodysplastic syndrome patients with or without HLA-DR15 allele

We studied the effects of the presence of the HLA-DR15 allele on the experimental and clinical features of myelodysplastic syndrome (MDS) by assessing the clinical data of 136 patients with MDS. We observed that the frequency of HLA-DR15 expression in MDS patients (38.7%) was significantly higher than that in the healthy controls (p < 0.01). We noted the following observations with regard to disease progression: None of the 46 HLA-DR15 positive patients with international prognostic scoring system (IPSS) scores [le]1 developed acute myeloid leukaemia (AML) during the follow-up period, while six of the 63 DR15-negative patients with the same IPSS score developed AML within a shorter follow-up period (p = 0.039). Furthermore, the incidence of poor chromosomal abnormalities, the percentage of patients with IPSS scores [ge]1.5 and the presence of [ge]5% blasts in the bone marrow in the DR15-positive patients were lower than the corresponding findings in the DR15-negative patients. In addition, we also recorded the following observations with regard to bone marrow (BM) failure: The bicytopenia/pancytopenia ratio in the DR15-positive patients was higher than that in the DR15-negative patients (92.4 vs. 78.3%; p = 0.029). The peripheral-neutrophil count and the platelet count in the DR15-positive patients were lower than those in the DR15-negative patients (p = 0.028 and p = 0.011, respectively). Moreover, hypocellularity was more easily detectable in the DR15-positive patients (26.4 vs. 16.9%). In addition, the BM CD4+ lymphocyte count and the CD4/CD8 ratio in the DR15-positive patients were higher than the corresponding values in the DR15-negative patients (p < 0.05 for both). However, there were no significant differences between the polarization of T-helper (Th) and T-cytotoxic (Tc) cells and the cytokine levels in these two patient groups. We concluded that the presence of the HLA-DR15 allele is indicative of a genetic susceptibility to MDS and, the presence of the HLA-DR15 allele showed less association with disease progression and greater association with BM failure. Copyright © 2009 John Wiley & Sons, Ltd.

Improved survival in red blood cell transfusion dependent patients with primary myelofibrosis (PMF) receiving iron chelation therapy

Many patients with primary myelofibrosis (PMF) become red blood cell (RBC) transfusion dependent (TD), risking iron overload (IOL). Iron chelation therapy (ICT) may decrease the risk of haemosiderosis associated organ dysfunction, though its benefit in PMF is undefined. To assess the effect of TD and ICT on survival in PMF, we retrospectively reviewed 41 patients. Clinical data were collected from the database and by chart review. The median age at PMF diagnosis was 64 (range 43-86) years. Median white blood cell (WBC) count at diagnosis was 7.6 (range 1.2-70.9) × 109/L; haemoglobin 104 (62-145) G/L; platelets 300 (38-2088) × 109/L. Lille, Strasser, Mayo and International Prognostic System (IPS) scores were: low risk, n = 15, 8, 11, 3; intermediate, n = 15, 19, 9, 16; high, n = 5, 11, 5, 7; respectively. Primary PMF treatment was: supportive care, n = 23; hydroxyurea, n = 10; immunomodulatory, n = 4; splenectomy, n = 2. Sixteen patients were RBC transfusion independent (TI) and 25 TD; of these 10 received ICT for a median of 18.3 (0.1-117) months. Pre-ICT ferritin levels were a median of 2318 (range 263-8400) and at follow up 1571 (1005-3211 µg/L (p = 0.01). In an analysis of TD patients, factors significant for overall survival (OS) were: WBC count at diagnosis (p = 0.002); monocyte count (p = 0.0001); Mayo score (p = 0.05); IPS (p = 0.02); number of RBC units (NRBCU) transfused (p = 0.02) and ICT (p = 0.003). In a multivariate analysis, significant factors were: NRBCU (p = 0.001) and ICT (p = 0.0001). Five year OS for TI, TD-ICT and TD-NO ICT were: 100, 89 and 34%, respectively (p = 0.003). The hazard ratio (HR) for receiving >20 RBCU was 7.6 (95% Confidence Intervals [CI] 1.2-49.3) and for ICT was 0.15 (0.03-0.77). In conclusion, 61% of PMF patients developed RBC-TD which portended inferior OS; however patients receiving ICT had comparatively improved OS, suggesting a clinical benefit. Prospective studies of IOL and the impact of ICT in PMF are warranted. Copyright © 2009 John Wiley & Sons, Ltd.

The therapeutic effect of rituximab on CD5-positive and CD5-negative diffuse large B-cell lymphoma

The prognosis of diffuse large B-cell lymphoma (DLBCL) has improved markedly in recent years of rituximab era. The prognosis of de novo CD5-positive DLBCL is reported to be poor, but the effect of rituximab on this type of lymphoma remains unclear. To investigate the effect of rituximab on CD5-positive DLBCL, we collected DLBCL patients and analysed prognostic factors. A total of 157 patients with DLBCL who were immunophenotyped with flow-cytometry (FCM) and treated with chemotherapy were subjected to analysis. Those treated with radiotherapy alone or with supportive therapy only were not included. Patients diagnosed in 2003 or later were treated with rituximab combined chemotherapy. There were 95 males and 62 females. Their age ranged from 20 to 91 years old, and the median was 65 years. Nineteen patients were diagnosed as having de novo CD5-positive DLBCL. Rituximab was given alongside chemotherapy in 85 patients. Of these, 11 were positive for CD5 and 74 were negative. The addition of rituximab improved the overall survival (OS) of DLBCL patients (2-year OS: 82% vs. 70%, p = 0.01). For CD5-negative DLBCL, patients treated with rituximab showed 2-year OS of 84%, which was significantly better than those treated without rituximab (70%, p = 0.008). However, for CD5-positive DLBCL, the prognosis was not statistically different between the patients treated with and without rituximab (59% vs. 50%, p = 0.72). Although rituximab improved the prognosis of DLBCL, such improvement was restricted to the CD5-negative group. Further investigation is required to improve the prognosis of patients with CD5-positive DLBCL. Copyright © 2009 John Wiley & Sons, Ltd.

MPL W515L/K mutations in 343 Chinese adults with JAK2V617F mutation-negative chronic myeloproliferative disorders detected by a newly developed RQ-PCR based on TaqMan MGB probes

Acquired mutations in the juxtamembrane region of MPL (W515L or W515K), the receptor for thrombopoietin, have been reported in patients with primary essential thrombocythemia (ET) or primary myelofibrosis (PMF). The mutations were detected by the newly developed real-time quantitative PCR (RQ-PCR) with TaqMan MGB probes and followed by the sequencing analysis. DNA samples were from 343 Chinese adults with JAK2V617F mutation-negative chronic myeloproliferative disorders (cMPDs). Reference curves were obtained using cloned fragments of MPL containing either the wild-type or MPL W515L or MPL W515K mutated sequence; the predicted sensitivity level was at least 0.5%(0.1-0.5%) for MPL W515L and 0.5%(0.2-0.5%) for MPL W515K mutant allele in a wild-type background. The detection rates of MPL W515 mutations were 3.5% in 199 ET patients (7/199), 12.5% in 24 PMF patients (3/24) and 5.6% in 36 cMPD-unclassed patients (2/36), respectively. No MPL W515 mutations were detected in 32 polycythemia vera (PV) patients, 40 chronic myeloid leukaemia (CML) patients, 12 hypereosinophilic syndrome (HES) patients and 29 normal volunteers. The mean calculated burden of MPL mutant alleles using RQ-PCR for MPL W515L/K was 24.88 ± 14.80% (range, 1.10-56.32%). MPL W515L/K patients presented lower haemoglobin levels, compared with the patients with JAK2V617F mutation-positive cMPDs (p < 0.01). The results demonstrated that RQ-PCR was a reliable and sensitive method for large-scale screening of the MPL W515L/K mutation in patients suspected to have a cMPD. It can also provide a quantitative estimate of mutant allele burden that might be useful for both patient prognosis and monitoring response to therapy. Copyright © 2009 John Wiley & Sons, Ltd.

Congenital factor XIII deficiency caused by two mutations in eight Tunisian families: molecular confirmation of a founder effect

Abstract Inherited factor XIII (FXIII) deficiency is a rare bleeding disorder characterized by an umbilical bleeding during the neonatal period, delayed soft tissue bruising, mucosal bleeding spontaneous intracranial hemorrhage, and soft tissue hemorrhages. Congenital FXIII deficiency is an autosomal recessive disorder, usually attributed to a defect in the FXIIIA and B subunits coding, respectively, by F13A and F13B genes. The aim of this study was to determine the molecular defects responsible for congenital factor XIII deficiency in eight Tunisian families. Molecular analysis was performed by direct DNA sequencing of polymerase chain reaction amplified fragments spanning the coding regions and splice junctions of the FXIIIA subunit gene (F13A) in probands and in families' members and compared with the reported sequence of this gene. In all patients, FXIIIA activity was undetectable and the FXIIIB was within the normal range. Direct sequencing of the F13A gene in all probands showed two mutations: the c.869insC mutation found in eight patients and the c.1226G > A transition found in only one. We also confirmed the presence of a founder effect for the first frequent mutation by using two microsatellite markers, HUMF13A01 and a generated ployAC marker (HUMF13A02). We describe here molecular abnormalities found in nine Tunisian probands diagnosed with FXIIIA deficiency. The identification of the founder mutation and polymorphisms allowed a genetic counseling in relatives of these families, and the antenatal diagnosis is now available. Content Type Journal ArticleCategory Original ArticleDOI 10.1007/s00277-009-0863-yAuthors Nacim Louhichi, Faculty of Medicine of Sfax Human Molecular Genetic Laboratory Avenue Magida Boulila 3029 Sfax TunisiaMoez Medhaffar, Hédi Chaker de Sfax Service of Hematology. C.H.U Sfax TunisiaIkhlass HadjSalem, Faculty of Medicine of Sfax Human Molecular Genetic Laboratory Avenue Magida Boulila 3029 Sfax TunisiaEmna Mkaouar-Rebai, Faculty of Medicine of Sfax Human Molecular Genetic Laboratory Avenue Magida Boulila 3029 Sfax TunisiaNourhene Fendri-Kriaa, Faculty of Medicine of Sfax Human Molecular Genetic Laboratory Avenue Magida Boulila 3029 Sfax TunisiaHouda Kanoun, Faculty of Medicine of Sfax Human Molecular Genetic Laboratory Avenue Magida Boulila 3029 Sfax TunisiaFiras Yaïch, Faculty of Medicine of Sfax Human Molecular Genetic Laboratory Avenue Magida Boulila 3029 Sfax TunisiaTawfik Souissi, Hédi Chaker de Sfax Service of Hematology. C.H.U Sfax TunisiaMoez Elloumi, Hédi Chaker de Sfax Service of Hematology. C.H.U Sfax TunisiaFaiza Fakhfakh, Faculty of Medicine of Sfax Human Molecular Genetic Laboratory Avenue Magida Boulila 3029 Sfax Tunisia Journal Annals of HematologyOnline ISSN 1432-0584Print ISSN 0939-5555

Salvage treatment with upfront melphalan 100 mg/m2 and consolidation with novel drugs for fulminant progression of multiple myeloma

Abstract Patients (pts) with fulminant progression (FPG) of multiple myeloma (MM) after autologous stem cell transplantation (ASCT) have poor prognosis. Pancytopenia, extramedullary disease, and/or renal impairment are often present, and treatment options are limited. We have retrospectively evaluated 31 pts with FPG of MM after ASCT who were treated upfront salvage therapy with melphalan 100 mg/m2 (MEL 100) followed by PBSC support and consolidation therapy using regimens containing thalidomide (n = 16) or bortezomib (n = 15). The overall response rate (ORR) was 58% (18/31). After MEL 100, one patient achieved complete remission (3%), 26% of pts very good partial remission, 29% of pts partial remission, and 42% of pts stable disease. Progression within 3 months after MEL 100 occurred in 35% of pts. The median follow-up from MEL 100 was 8 months. The median TTP was 5 months (range, 2–15 months), and the median OS was 8 months (range, 3–23 months). There were no treatment-related deaths. In fulminant progression of MM, upfront MEL 100 is a safe salvage regimen with good response rate (ORR, 58%). Treatment with upfront MEL 100 followed by a thalidomide- or bortezomib-based regimen can prolong overall survival to more than 12 months in 33% of pts with fulminant progression of MM. Content Type Journal ArticleCategory Original ArticleDOI 10.1007/s00277-009-0862-zAuthors Marta Krejci, Masaryk University Hospital Department of Internal Medicine–Hematooncology Jihlavska 20 Brno 625 00 Czech RepublicZdenek Adam, Masaryk University Hospital Department of Internal Medicine–Hematooncology Jihlavska 20 Brno 625 00 Czech RepublicTomas Buchler, Thomayer University Hospital 1st Faculty of Medicine, Department of Oncology Prague Czech RepublicAndrea Krivanova, Masaryk University Hospital Department of Internal Medicine–Hematooncology Jihlavska 20 Brno 625 00 Czech RepublicLudek Pour, Masaryk University Hospital Department of Internal Medicine–Hematooncology Jihlavska 20 Brno 625 00 Czech RepublicLenka Zahradova, Masaryk University Hospital Department of Internal Medicine–Hematooncology Jihlavska 20 Brno 625 00 Czech RepublicMichal Holanek, Masaryk University Hospital Department of Internal Medicine–Hematooncology Jihlavska 20 Brno 625 00 Czech RepublicViera Sandecka, Masaryk University Hospital Department of Internal Medicine–Hematooncology Jihlavska 20 Brno 625 00 Czech RepublicJiri Mayer, Masaryk University Hospital Department of Internal Medicine–Hematooncology Jihlavska 20 Brno 625 00 Czech RepublicJiri Vorlicek, Masaryk University Hospital Department of Internal Medicine–Hematooncology Jihlavska 20 Brno 625 00 Czech RepublicRoman Hajek, Masaryk University Hospital Department of Internal Medicine–Hematooncology Jihlavska 20 Brno 625 00 Czech Republic Journal Annals of HematologyOnline ISSN 1432-0584Print ISSN 0939-5555

Clinical efficacy of a bortezomib, cyclophosphamide, thalidomide, and dexamethasone (Vel-CTD) regimen in patients with relapsed or refractory multiple myeloma: a phase II study

Abstract The clinical efficacy and safety of a four-drug combination of bortezomib, cyclophosphamide, thalidomide, and dexamethasone was assessed for patients with relapsed or refractory multiple myeloma. Seventy patients received at least two cycles of treatment with bortezomib 1.3 mg/m2 intravenously on days 1, 4, 8, and 11; cyclophosphamide 150 mg/m2 orally on days 1–4; thalidomide 50 mg/day orally every day; and dexamethasone 20 mg/m2 intravenously on days 1, 4, 8, and 11. The overall best response rate was 88%, with 46% complete response, 9% very good partial response, and 33% partial response. After a median follow-up of 12.6 months, the median progression-free survival (PFS) was 14.6 months with a 3-year PFS of 14% and the median overall survival (OS) was 31.6 months with a 3-year OS of 47%. Grade 3 or 4 adverse events included thrombocytopenia (12%), neutropenia (4%), peripheral sensory neuropathy (3%), with thrombosis being very rare (<1%). Bortezomib combined with cyclophosphamide, thalidomide, and dexamethasone is a highly effective salvage therapy with manageable toxicity for patients with relapsed or refractory multiple myeloma. Content Type Journal ArticleCategory Original ArticleDOI 10.1007/s00277-009-0856-xAuthors Yeo-Kyeoung Kim, Chonnam National University Medical School Gwangju Republic of KoreaSang-Kyun Sohn, Kyungpook National University Medical School Daegu Republic of KoreaJae-Hoon Lee, Gachon University Incheon Republic of KoreaDeok-Hwan Yang, Chonnam National University Medical School Gwangju Republic of KoreaJoon-Ho Moon, Kyungpook National University Medical School Daegu Republic of KoreaJae-Sook Ahn, Chonnam National University Medical School Gwangju Republic of KoreaHyeoung-Joon Kim, Chonnam National University Medical School Gwangju Republic of KoreaJe-Jung Lee, Chonnam National University Medical School Gwangju Republic of KoreaThe Korean Multiple Myeloma Working Party (KMMWP), Center for Biomedical Human Resources at Chonnam National University The Brain Korea 21 Project Gwangju Republic of Korea Journal Annals of HematologyOnline ISSN 1432-0584Print ISSN 0939-5555

Erratum to: Results of FLT3 mutation screening and correlations with immunophenotyping in 169 Brazilian patients with acute myeloid leukemia

Erratum to: Results of FLT3 mutation screening and correlations with immunophenotyping in 169 Brazilian patients with acute myeloid leukemia Content Type Journal ArticleCategory ErratumDOI 10.1007/s00277-009-0860-1Authors Antonio R. Lucena-Araujo, University of São Paulo Hematology Division, Department of Internal Medicine, National Institute of Science and Technology on Cell Based Therapy, Medical School of Ribeirão Preto Ribeirão Preto BrazilDanielle L. Souza, University of São Paulo Hematology Division, Department of Internal Medicine, National Institute of Science and Technology on Cell Based Therapy, Medical School of Ribeirão Preto Ribeirão Preto BrazilFabio Morato de Oliveira, University of São Paulo Hematology Division, Department of Internal Medicine, National Institute of Science and Technology on Cell Based Therapy, Medical School of Ribeirão Preto Ribeirão Preto BrazilMariana Tereza Lira Benicio, University of São Paulo Hematology Division, Department of Internal Medicine, National Institute of Science and Technology on Cell Based Therapy, Medical School of Ribeirão Preto Ribeirão Preto BrazilLorena L. Figueiredo-Pontes, University of São Paulo Hematology Division, Department of Internal Medicine, National Institute of Science and Technology on Cell Based Therapy, Medical School of Ribeirão Preto Ribeirão Preto BrazilBarbara A. Santana-Lemos, University of São Paulo Hematology Division, Department of Internal Medicine, National Institute of Science and Technology on Cell Based Therapy, Medical School of Ribeirão Preto Ribeirão Preto BrazilGuilherme A. dos Santos, University of São Paulo Hematology Division, Department of Internal Medicine, National Institute of Science and Technology on Cell Based Therapy, Medical School of Ribeirão Preto Ribeirão Preto BrazilRafael H. Jacomo, University of São Paulo Hematology Division, Department of Internal Medicine, National Institute of Science and Technology on Cell Based Therapy, Medical School of Ribeirão Preto Ribeirão Preto BrazilAnemari R. Dinarte-Santos, University of São Paulo Department of Genetics, National Institute of Science and Technology on Cell Based Therapy, Medical School of Ribeirão Preto Av. Bandeirantes, 3900 14048-900 Ribeirão Preto São Paulo BrazilMihoko Yamamoto, Federal University of São Paulo Department of Hematology and Hemotherapy São Paulo BrazilWilson A. Silva-Jr, University of São Paulo Department of Genetics, National Institute of Science and Technology on Cell Based Therapy, Medical School of Ribeirão Preto Av. Bandeirantes, 3900 14048-900 Ribeirão Preto São Paulo BrazilMaria de Lourdes Chauffaille, Federal University of São Paulo Department of Hematology and Hemotherapy São Paulo BrazilEduardo M. Rego, University of São Paulo Hematology Division, Department of Internal Medicine, National Institute of Science and Technology on Cell Based Therapy, Medical School of Ribeirão Preto Ribeirão Preto Brazil Journal Annals of HematologyOnline ISSN 1432-0584Print ISSN 0939-5555

Hyperlipidemic myeloma: review of 53 cases

Abstract Hyperlipidemic myeloma is a rare and poorly understood variant of multiple myeloma. We report the case of a 53-year-old woman with hyperlipidemic myeloma, skin xanthomas and hyperviscosity syndrome who underwent allogeneic bone marrow transplantation. A comprehensive literature search identified 52 additional cases with plasma cell disease and hyperlipidemia. A detailed analysis revealed several characteristics of these patients as compared to multiple myeloma with normal lipid status: (1) IgA paraprotein was present in the majority (53% vs. 21% in classical multiple myeloma). (2) Skin xanthomas, especially in the palmar creases, elbows, and knees were common (70%). (3) Hyperviscosity syndrome occurred more often (26% vs. 2–6%). While conventional lipid-lowering therapy had only marginal effects, successful anti-myeloma therapy also reduced hyperlipidemia. Analyses of the mechanisms leading to hyperlipidemia documented complexes of paraprotein and lipoprotein in 75% of the 32 cases tested, suggesting an inhibitory role of the paraprotein on lipid degradation. In conclusion, the clinical characteristics, the therapeutic options, and the pathophysiologic mechanisms of hyperlipidemic myeloma are comprehensively reported using the available data from all 53 published cases in the literature. Content Type Journal ArticleCategory Original ArticleDOI 10.1007/s00277-009-0849-9Authors Benjamin Misselwitz, University Hospital Zürich Department of Internal Medicine Rämistr. 100 8091 Zürich SwitzerlandJeroen S. Goede, University Hospital Zürich Clinic of Haematology Rämistr. 100 8091 Zürich SwitzerlandBernhard C. Pestalozzi, University Hospital Zürich Clinic of Oncology Rämistr. 100 8091 Zürich SwitzerlandUrs Schanz, University Hospital Zürich Clinic of Haematology Rämistr. 100 8091 Zürich SwitzerlandJörg D. Seebach, University Hospital Zürich Department of Internal Medicine Rämistr. 100 8091 Zürich Switzerland Journal Annals of HematologyOnline ISSN 1432-0584Print ISSN 0939-5555

Cytogenetic features and prognosis analysis in Chinese patients with myelodysplastic syndrome: a multicenter study

Abstract It has been suggested that Asian and Western myelodysplastic syndrome (MDS) patients have different cytogenetic and prognostic features. In this study, we retrospectively analyzed clinical and cytogenetic data from 435 Chinese adult primary MDS patients. In addition, we evaluated the prognostic value of the World Health Organization classification as well as six prognostic scoring systems in these patients. The median follow-up time was 25.1 months (5.5–53.2). Of the 435 patients, 186 (42.8%) had died and 40 (9.2%) had progressed to acute myeloid leukemia. Multivariate analysis identified older age, higher percent of marrow blasts, and poor-risk IPSS cytogenetics as characteristics associated with worse survival and higher risk of leukemia transformation. Low platelets, hemoglobin, and mean corpuscular volume were independent factors associated only with worse survival. Among the 424 patients in whom the results of cytogenetic analyses were available, 164 (38.7%) showed karyotypic abnormalities. Incidence of trisomy 8 was common but sole del(5q) was rare in Chinese MDS patients. For predicting survival, most scoring systems were meaningful for stratifying patients into different subgroups, with the exception of the WPSS scoring system. For predicting leukemia evolution, the Spanish scoring system was most effective. Patients with RAEB-2 showed different prognoses from those with RAEB-1. However, there was no significant difference in prognoses between patients with refractory cytopenia with multilineage dysplasia (RCMD) from RA or RARS. In summary, this analysis indicated the presence of a different cytogenetic pattern as well as prognostic features in Chinese MDS patients. Content Type Journal ArticleCategory Original ArticleDOI 10.1007/s00277-009-0861-0Authors Hong Wang, Huashan Hospital of Fudan University Department of Haematology 12 Wulumuqi Road central Shanghai 200040 ChinaXiaoQin Wang, Huashan Hospital of Fudan University Department of Haematology 12 Wulumuqi Road central Shanghai 200040 ChinaXiaoPing Xu, Huashan Hospital of Fudan University Department of Haematology 12 Wulumuqi Road central Shanghai 200040 ChinaGuoWei Lin, Huashan Hospital of Fudan University Department of Haematology 12 Wulumuqi Road central Shanghai 200040 China Journal Annals of HematologyOnline ISSN 1432-0584Print ISSN 0939-5555

ABO discrepancy in an elderly patient with IgA kappa-type multiple myeloma

ABO discrepancy in an elderly patient with IgA kappa-type multiple myeloma Content Type Journal ArticleCategory Letter to the EditorDOI 10.1007/s00277-009-0858-8Authors So Young Kim, Kyung Hee University School of Medicine Department of Laboratory Medicine 1 Hoegi-dong, Dongdaemun-gu Seoul 130-702 Republic of KoreaSeung Hwan Oh, Inje University College of Medicine Department of Laboratory Medicine Gaegeum-dong, Busanjin-gu Busan 614-735 Republic of KoreaKyung Sun Park, Kyung Hee University School of Medicine Department of Laboratory Medicine 1 Hoegi-dong, Dongdaemun-gu Seoul 130-702 Republic of KoreaMin Jin Kim, Kyung Hee University School of Medicine Department of Laboratory Medicine 1 Hoegi-dong, Dongdaemun-gu Seoul 130-702 Republic of KoreaGayoung Lim, Kyung Hee University School of Medicine Department of Laboratory Medicine 1 Hoegi-dong, Dongdaemun-gu Seoul 130-702 Republic of KoreaSun Young Cho, Kyung Hee University School of Medicine Department of Laboratory Medicine 1 Hoegi-dong, Dongdaemun-gu Seoul 130-702 Republic of KoreaHee Joo Lee, Kyung Hee University School of Medicine Department of Laboratory Medicine 1 Hoegi-dong, Dongdaemun-gu Seoul 130-702 Republic of KoreaJin-Tae Suh, Kyung Hee University School of Medicine Department of Laboratory Medicine 1 Hoegi-dong, Dongdaemun-gu Seoul 130-702 Republic of KoreaHwi-Joong Yoon, Kyung Hee University School of Medicine Department of Hematology–Oncology 1 Hoegi-dong, Dongdaemun-gu Seoul 130-702 Republic of KoreaTae Sung Park, Kyung Hee University School of Medicine Department of Laboratory Medicine 1 Hoegi-dong, Dongdaemun-gu Seoul 130-702 Republic of Korea Journal Annals of HematologyOnline ISSN 1432-0584Print ISSN 0939-5555

Jumping translocation involving 1q21 during long-term complete remission of acute myeloid leukemia

Jumping translocation involving 1q21 during long-term complete remission of acute myeloid leukemia Content Type Journal ArticleCategory Letter to the EditorDOI 10.1007/s00277-009-0855-yAuthors Sumimasa Nagai, University of Tokyo Department of Hematology and Oncology, Graduate School of Medicine 7-3-1, Hongo, Bunkyo-ku Tokyo 1138655 JapanYasuhito Nannya, University of Tokyo Department of Hematology and Oncology, Graduate School of Medicine 7-3-1, Hongo, Bunkyo-ku Tokyo 1138655 JapanTsuyoshi Takahashi, University of Tokyo Department of Hematology and Oncology, Graduate School of Medicine 7-3-1, Hongo, Bunkyo-ku Tokyo 1138655 JapanMineo Kurokawa, University of Tokyo Department of Hematology and Oncology, Graduate School of Medicine 7-3-1, Hongo, Bunkyo-ku Tokyo 1138655 Japan Journal Annals of HematologyOnline ISSN 1432-0584Print ISSN 0939-5555

Acquired erythropoietic protoporphyria

Acquired erythropoietic protoporphyria Content Type Journal ArticleCategory Letter to the EditorDOI 10.1007/s00277-009-0859-7Authors Daniel Blagojevic, Landesklinikum St. Poelten Karl Landsteiner Institute for Dermatological Research St. Poelten AustriaThomas Schenk, Landesklinikum St. Poelten Department of Internal Medicine I St. Poelten AustriaOskar Haas, Ambulatorium Medgen Vienna AustriaBrigitte Zierhofer, Landesklinikum St. Poelten Karl Landsteiner Institute for Dermatological Research St. Poelten AustriaChristophoros Konnaris, Medical University of Vienna Department of Gynecology and Obstetrics Vienna AustriaFranz Trautinger, Landesklinikum St. Poelten Karl Landsteiner Institute for Dermatological Research St. Poelten Austria Journal Annals of HematologyOnline ISSN 1432-0584Print ISSN 0939-5555

Resolution of invasive fungal sinusitis in immunocompromised patients: neutrophil count is crucial beside a combined medical and surgical approach

Resolution of invasive fungal sinusitis in immunocompromised patients: neutrophil count is crucial beside a combined medical and surgical approach Content Type Journal ArticleCategory Letter to the EditorDOI 10.1007/s00277-009-0854-zAuthors Patrizia Zappasodi, Foundation IRCCS Policlinico San Matteo, University of Pavia Division of Haematology 27100 Pavia ItalyMarianna Rossi, Foundation IRCCS Policlinico San Matteo, University of Pavia Division of Haematology 27100 Pavia ItalyCarlo Castagnola, Foundation IRCCS Policlinico San Matteo, University of Pavia Division of Haematology 27100 Pavia ItalyFabio Pagella, Foundation IRCCS Policlinico San Matteo, University of Pavia Department of Otorhinolaryngology Pavia ItalyElina Matti, Foundation IRCCS Policlinico San Matteo, University of Pavia Department of Otorhinolaryngology Pavia ItalyCaterina Cavanna, Foundation IRCCS Policlinico San Matteo, University of Pavia Virology and Microbiology Laboratory, Department of Diagnostic Medicine and Services Pavia ItalyAlessandro Corso, Foundation IRCCS Policlinico San Matteo, University of Pavia Division of Haematology 27100 Pavia ItalyMaurizio Bonfichi, Foundation IRCCS Policlinico San Matteo, University of Pavia Division of Haematology 27100 Pavia ItalyMario Lazzarino, Foundation IRCCS Policlinico San Matteo, University of Pavia Division of Haematology 27100 Pavia Italy Journal Annals of HematologyOnline ISSN 1432-0584Print ISSN 0939-5555

Amyloid in bone marrow smears of patients affected by multiple myeloma

Abstract Systemic AL amyloidosis is associated with nearly 15% of cases of multiple myeloma, but data on the frequency and significance of amyloid deposits in the bone marrow of patients affected by multiple myeloma without clinical signs of systemic amyloidosis are scanty. Bone marrow smears of 166 unselected patients affected by multiple myeloma (126 at diagnosis and 40 after treatment) were stained with Congo red and studied by transmission and birefringence microscopy. Both focal and diffuse storages were considered positive. Overall, 67 patients were positive and 99 were negative to Congo red and apple-green birefringence. In particular, 51 of the 126 patients studied at diagnosis and 16 of the 40 patients with advanced disease were positive. Seventeen patients were reassessed after a mean follow-up of 32 months (range: 6–91): disappearance of amyloid deposits was verified in three cases, all responsive to bortezomib-based regimens. The preliminary data available suggest that amyloid deposition in the marrow of myeloma patients is frequent, as it can be traced in nearly 40% of cases. We failed to find correlations between bone marrow amyloid deposits and immunoglobulin type, disease stage, plasma cells percentage, hemoglobin, calcium, creatinine, albumin, or β2microglobulin. Significantly higher incidence of moderate/severe peripheral neuropathy was found in patients with marrow amyloid exposed to potentially neurotoxic antineoplastic agents. Further studies and prolonged follow-up are needed to validate our findings and to define possible prognostic aspects. Content Type Journal ArticleCategory Original ArticleDOI 10.1007/s00277-009-0857-9Authors Fara Petruzziello, University Federico II Hematology, Department of Biochemistry and Medical Biotechnology Naples ItalyPio Zeppa, University Federico II Department of Anatomopathology and Cytopathology Naples ItalyLucio Catalano, University Federico II Hematology, Department of Biochemistry and Medical Biotechnology Naples ItalyImmacolata Cozzolino, University Federico II Department of Anatomopathology and Cytopathology Naples ItalyGiuseppe Gargiulo, Stazione Zoologica Anton Dhorn Naples ItalyPellegrino Musto, Hematology and Stem Cell Transplantation Unit, IRCCS Rionero in Vulture PZ ItalyFiorella D’Auria, Hematology and Stem Cell Transplantation Unit, IRCCS Rionero in Vulture PZ ItalyVincenzo Liso, Hematology, AOUCP Bari Bari ItalyRita Rizzi, Hematology, AOUCP Bari Bari ItalyNadia Caruso, Hematology Unit, Annunziata Hospital Cosenza ItalyCatello Califano, PO Umberto I Department of Oncology and Hematology Nocera Inferiore ItalyEugenio Piro, Hematology Unit, AO Pugliese-Ciaccio Catanzaro ItalyMaurizio Musso, Oncohematology Unit, Oncology Department La Maddalena Palermo ItalyVincenza Bonanno, Oncohematology Unit, Oncology Department La Maddalena Palermo ItalyAntonietta Pia Falcone, IRCCS Casa Sollievo della Sofferenza Department of Hematology S. Giovanni Rotondo FG ItalySalvatore Tafuto, Oncohematology, PO S.Maria delle Grazie Pozzuoli ItalyFrancesco Di Raimondo, AO Ferrarotto Department of Hematology Catania ItalyMichelino De Laurentiis, University Federico II Department of Endocrinology and Clinical Oncology Naples ItalyFabrizio Pane, University Federico II Hematology, Department of Biochemistry and Medical Biotechnology Naples ItalyLucio Palombini, University Federico II Department of Anatomopathology and Cytopathology Naples ItalyBruno Rotoli, University Federico II Hematology, Department of Biochemistry and Medical Biotechnology Naples Italy Journal Annals of HematologyOnline ISSN 1432-0584Print ISSN 0939-5555

Splenic artery pseudoaneurysm with rupture by transformed splenic marginal zone B cell lymphoma

Splenic artery pseudoaneurysm with rupture by transformed splenic marginal zone B cell lymphoma Content Type Journal ArticleCategory Letter to the EditorDOI 10.1007/s00277-009-0851-2Authors Yu-Chung Huang, Taipei Veterans General Hospital Division of Hematology & Oncology, Department of Medicine No. 201, Sec. 2, Shipai Rd Taipei 112 TaiwanZhi-Yi Xie, Taipei Veterans General Hospital Division of Hematology & Oncology, Department of Medicine No. 201, Sec. 2, Shipai Rd Taipei 112 TaiwanHsiou-Shan Tseng, Taipei Veterans General Hospital Department of Radiology No. 201, Sec. 2, Shipai Rd Taipei 112 TaiwanChing-Feng Yang, Taipei Veterans General Hospital Department of Pathology No. 201, Sec. 2, Shipai Rd Taipei 112 TaiwanLiang-Tsai Hsiao, Taipei Veterans General Hospital Division of Hematology & Oncology, Department of Medicine No. 201, Sec. 2, Shipai Rd Taipei 112 Taiwan Journal Annals of HematologyOnline ISSN 1432-0584Print ISSN 0939-5555

Impact of critical care reconfiguration and track-and-trigger outreach team intervention on outcomes of haematology patients requiring intensive care admission

Abstract Patients with haematological disorders have previously been considered to have poor outcomes following admission to intensive care units. Although a number of haematology centres from outside the UK have now demonstrated improved outcomes, the continuing perception of poor outcomes in this patient group continues to adversely affect their chances of being admitted to some intensive care units (ICUs). Over the past 10 years, there have been many advances within the disciplines of both haematology and intensive care medicine. This study was done to assess outcomes and the impact of an early warning scoring system (EWS) and early involvement of ICU outreach teams. One hundred five haematology patients (haematopoietic stem cell transplant (HSCT) or non-HSCT) had 114 admissions to ICU between April 2006 and August 2008 which coincided with hospital-wide implementation of EWS. The survival to ICU discharge was 56 (53%). Thirty-three (33%) patients were alive at 6 months giving a 1-year survival of 31%. Of the 39 HSCT patients, nine were post-autologous and 30 post-allogeneic transplant. The survival to ICU discharge was 22 (56%) with 14 (36%) patients alive at 6 months. One year survival was 36%. Prior to the introduction of EWS and critical care outreach team (2004), survival to ICU discharge was 44% which has increased to 53% (2006–2008). This is despite an increase in mechanical ventilation in 2006–2008 (50%) as compared to 2004 (32%).The improvement in ICU survivorship was even more prominent in HSCT patients (37% in 2004 versus 56% in 2006–2008). There was a trend towards decreasing Acute Physiology and Chronic Health Evaluation scores with time, suggesting appropriate patients being identified earlier and having timely escalation of their treatment. Content Type Journal ArticleCategory Original ArticleDOI 10.1007/s00277-009-0853-0Authors Syed W. I. Bokhari, Nottingham University Hospitals—City Campus Department of Clinical Haematology Nottingham UKTalha Munir, Nottingham University Hospitals—City Campus Department of Clinical Haematology Nottingham UKShabeeha Memon, Nottingham University Hospitals—City Campus Department of Clinical Haematology Nottingham UKJenny L. Byrne, Nottingham University Hospitals—City Campus Department of Clinical Haematology Nottingham UKNigel H. Russell, Nottingham University Hospitals—City Campus Department of Clinical Haematology Nottingham UKMartin Beed, Nottingham University Hospitals—City Campus Critical Care Department Nottingham UK Journal Annals of HematologyOnline ISSN 1432-0584Print ISSN 0939-5555

Patients with del(5q) MDS who fail to achieve sustained erythroid or cytogenetic remission after treatment with lenalidomide have an increased risk for clonal evolution and AML progression

Abstract Lenalidomide consistently induces transfusion independence and complete cytogenetic response in patients with myelodysplastic syndromes with 5q deletion. Only limited information on long-term outcome is currently available. We performed a long-term follow-up analysis of 42 patients with low or intermediate risk myelodysplastic syndromes and 5q deletion treated with lenalidomide. At a median follow-up of 40 months, 58% of the patients achieved an erythroid response and 48% a cytogenetic response. Thirty-six percent of patients progressed into acute myeloid leukaemia. Most of them (87%) acquired chromosome aberrations in addition to the 5q deletion, i.e. underwent clonal evolution during leukaemogenesis. There were no clinical, cytological or cytogenetic markers at study entry that allowed prediction of increased risk of leukaemic transformation. However, erythroid and cytogenetic responders had a significantly decreased risk of progression to acute myeloid leukaemia (p = 0.001 and p = 0.009, respectively) compared to non-responders. Three and 5 years after study entry, the cumulative incidence of acute myeloid leukaemia for patients with a cytogenetic response was 10% and 21%, respectively, and for patients without cytogenetic response, it was 46% and 60%. Patients with del(5q) myelodysplastic syndromes without erythroid or cytogenetic remission after treatment with lenalidomide have a high risk for clonal evolution and acute myeloid leukaemia progression. In refractory, or relapsing, patients, genetic instability and clonal evolution seem to be the driving forces of leukaemic transformation. Regular follow-up investigations of del(5q) myelodysplastic syndrome patients treated with lenalidomide may help to identify patients requiring alternative treatment strategies. Content Type Journal ArticleCategory Original ArticleDOI 10.1007/s00277-009-0846-zAuthors Gudrun Göhring, Hannover Medical School Institute of Cell and Molecular Pathology Carl-Neuberg-Str. 1 30625 Hannover GermanyAristoteles Giagounidis, St. Johannes Hospital Department of Haematology, Oncology and Clinical Immunology Duisburg GermanyGuntram Büsche, Hannover Medical School Institute of Pathology Hannover GermanyHans Heinrich Kreipe, Hannover Medical School Institute of Pathology Hannover GermanyMartin Zimmermann, Hannover Medical School Department of Paediatric Haematology/Oncology Hannover GermanyEva Hellström-Lindberg, Karolinska University Hospital Division of Haematology and Centre of Experimental Haematology, Department of Medicine, Karolinska Institutet Stockholm SwedenCarlo Aul, St. Johannes Hospital Department of Haematology, Oncology and Clinical Immunology Duisburg GermanyBrigitte Schlegelberger, Hannover Medical School Institute of Cell and Molecular Pathology Carl-Neuberg-Str. 1 30625 Hannover Germany Journal Annals of HematologyOnline ISSN 1432-0584Print ISSN 0939-5555

Serum albumin level is a significant prognostic factor reflecting disease severity in symptomatic multiple myeloma

Abstract Serum albumin level, in association with serum interleukin-6 level, is a significant prognostic factor in multiple myeloma patients. The aim of this study was to determine any clinical factors associated with decreased serum albumin level. We retrospectively reviewed the records of 373 patients diagnosed with multiple myeloma at the Asan Medical Center, Seoul, Korea, between January 1996 and March 2008. Patients were divided into two groups according to serum albumin level (above or below 3.5 g/dL, the prognostic cutoff value), and clinical parameters were compared between groups. We aimed to identify any clinical parameters associated with low serum albumin levels. The group with serum albumin <3.5 g/dL showed older patient age, lower hemoglobin level, and poorer performance status. By contrast, levels of serum beta2-microglobulin, serum M protein, and bone marrow plasma cells were significantly higher in the group with low serum albumin levels. No significant between-group differences were found when serum calcium and creatinine levels were compared. Numbers of cytogenetic abnormalities and lytic bone lesions also showed no significant between-group differences. In both univariate and multivariate analyses, serum albumin level less than 3.5 g/dL was identified as a significant pretreatment prognostic factor. Lower serum albumin levels in multiple myeloma patients are associated with clinical factors reflecting disease severity. Content Type Journal ArticleCategory Original ArticleDOI 10.1007/s00277-009-0841-4Authors Jeong Eun Kim, University of Ulsan College of Medicine Department of Oncology, Asan Medical Center 86 Asan Byeongwon gil, Songpa-gu Seoul 138-736 South KoreaChanghoon Yoo, University of Ulsan College of Medicine Department of Oncology, Asan Medical Center 86 Asan Byeongwon gil, Songpa-gu Seoul 138-736 South KoreaDae Ho Lee, University of Ulsan College of Medicine Department of Oncology, Asan Medical Center 86 Asan Byeongwon gil, Songpa-gu Seoul 138-736 South KoreaSang-We Kim, University of Ulsan College of Medicine Department of Oncology, Asan Medical Center 86 Asan Byeongwon gil, Songpa-gu Seoul 138-736 South KoreaJung-Shin Lee, University of Ulsan College of Medicine Department of Oncology, Asan Medical Center 86 Asan Byeongwon gil, Songpa-gu Seoul 138-736 South KoreaCheolwon Suh, University of Ulsan College of Medicine Department of Oncology, Asan Medical Center 86 Asan Byeongwon gil, Songpa-gu Seoul 138-736 South Korea Journal Annals of HematologyOnline ISSN 1432-0584Print ISSN 0939-5555

ABO discrepancy in a young Korean serviceman with common variable immunodeficiency

ABO discrepancy in a young Korean serviceman with common variable immunodeficiency Content Type Journal ArticleCategory Letter to the EditorDOI 10.1007/s00277-009-0840-5Authors Seung Hwan Oh, Inje University Department of Laboratory Medicine, College of Medicine GaeGeum-dong, Busanjin-gu Busan 614-735 South KoreaCheol-In Kang, Sungkyunkwan University School of Medicine Division of Infectious Diseases, Samsung Medical Center 50 Ilwon-dong, Gangnam-gu Seoul 135-710 South KoreaJuwon Kim, Yonsei University College of Medicine Department of Laboratory Medicine 250 Seongsanno, Seodaemun-gu Seoul 120-752 South KoreaTae Sung Park, Kyung Hee University School of Medicine Department of Laboratory Medicine 1 Hoegi-dong, Dongdaemun-gu Seoul 130-702 South Korea Journal Annals of HematologyOnline ISSN 1432-0584Print ISSN 0939-5555

Plasmablastic crisis of Philadelphia chromosome-positive chronic myeloid leukemia

Plasmablastic crisis of Philadelphia chromosome-positive chronic myeloid leukemia Content Type Journal ArticleCategory Letter to the EditorDOI 10.1007/s00277-009-0852-1Authors Kalliopi N. Manola, National Center for Scientific Research (NCSR) “Demokritos” Laboratory of Cytogenetics 15310 Aghia Paraskevi Athens GreeceDespina Pantelidou, Medical School AUTH First Department of Internal Medicine, Hematology department Thessaloniki GreeceMaria Papaioannou, Medical School AUTH First Department of Internal Medicine, Hematology department Thessaloniki Greece Journal Annals of HematologyOnline ISSN 1432-0584Print ISSN 0939-5555

18F-FDG PET/CT in primary non-Hodgkin's lymphoma of the sinonasal tract

18F-FDG PET/CT in primary non-Hodgkin's lymphoma of the sinonasal tract Content Type Journal ArticleCategory Letter to the EditorDOI 10.1007/s00277-009-0842-3Authors Adrian Tempescul, CHU Brest Department of Clinical Hematology, Institute of Cancerology and Hematology Avenue Foch 29609 Brest FranceSolene Querellou, CHU Brest Department of Nuclear Medicine Avenue Foch 29609 Brest FranceJean-Christophe Ianotto, CHU Brest Department of Clinical Hematology, Institute of Cancerology and Hematology Avenue Foch 29609 Brest FranceSylvie Boisramé, CHU Brest Department of Odontology Avenue Foch 29609 Brest FranceGerald Valette, CHU Brest Department of ORL Avenue Foch 29609 Brest FranceChristian Berthou, CHU Brest Department of Clinical Hematology, Institute of Cancerology and Hematology Avenue Foch 29609 Brest France Journal Annals of HematologyOnline ISSN 1432-0584Print ISSN 0939-5555

Vitamin D deficiency and anemia: a cross-sectional study

Abstract Vitamin D has been suggested to have an effect on erythropoiesis. We sought to evaluate the prevalence of anemia in a population of individuals with vitamin D deficiency compared with those with normal levels in a population of a large integrated healthplan. A cross-sectional analysis in the period 1 January 2004 through 31 December 2006 of subjects with documented concurrent levels of 25-hydroxyvitamin D and hemoglobin were evaluated. Vitamin D deficiency was defined as <30 ng/mL and anemia was defined as a hemoglobin <11 g/dL. A total of 554 subjects were included in the analysis. Anemia was present in 49% of 25-hydroxyvitamin D-deficient subjects compared with 36% with normal 25-hydroxyvitamin D levels (p < 0.01). Odds ratio for anemia in subjects with 25-hydroxyvitamin D deficiency using logistic regressions and controlling for age, gender, and chronic kidney disease was 1.9 (95% CI 1.3–2.7). 25-hydroxyvitamin D-deficient subjects had a lower mean Hb (11.0 vs. 11.7; p = 0.12 ) and a higher prevalence of erythrocyte stimulating agent use (47% vs. 24%; p < 0.05). This study demonstrates an association of vitamin D deficiency and a greater risk of anemia, lower mean hemoglobin, and higher usage of erythrocyte-stimulating agents. Future randomized studies are warranted to examine whether vitamin D directly affects erythropoiesis. Content Type Journal ArticleCategory Original ArticleDOI 10.1007/s00277-009-0850-3Authors John J. Sim, Kaiser Permanente Los Angeles Medical Center Division of Nephrology and Hypertension 4700 Sunset Blvd., 2nd Floor Nephrology Los Angeles CA 90027 USAPeter T. Lac, Kaiser Permanente Los Angeles Medical Center Division of Nephrology and Hypertension 4700 Sunset Blvd., 2nd Floor Nephrology Los Angeles CA 90027 USAIn Lu A. Liu, Kaiser Permanente Southern California Department of Research and Evaluation 100 S. Los Robles Av Pasadena CA 91107 USASamuel O. Meguerditchian, Kaiser Permanente Los Angeles Medical Center Division of Nephrology and Hypertension 4700 Sunset Blvd., 2nd Floor Nephrology Los Angeles CA 90027 USAVictoria A. Kumar, Kaiser Permanente Los Angeles Medical Center Division of Nephrology and Hypertension 4700 Sunset Blvd., 2nd Floor Nephrology Los Angeles CA 90027 USADean A. Kujubu, Kaiser Permanente Los Angeles Medical Center Division of Nephrology and Hypertension 4700 Sunset Blvd., 2nd Floor Nephrology Los Angeles CA 90027 USAScott A. Rasgon, Kaiser Permanente Los Angeles Medical Center Division of Nephrology and Hypertension 4700 Sunset Blvd., 2nd Floor Nephrology Los Angeles CA 90027 USA Journal Annals of HematologyOnline ISSN 1432-0584Print ISSN 0939-5555

Glutathione S-transferase gene deletions and their effect on iron status in HbE/β thalassemia patients

Abstract Iron overload and oxidative stress are main pathophysiological features of HbE/β thalassemia patients. Glutathione S-transferase genes (GSTT1 and GSTM1) are well known detoxification agents, and any mutation in the gene is known to cause oxidative damage. This study was aimed to compare the prevalence of GST deletions in 240 HbE/β thalassemia patients with 100 controls and to determine role of deletions on iron overload. We observed significantly higher frequency of GSTT1 (P = 0.001) and GSTT1/GSTM1 (P = 0.03) in comparison to controls. Patients who had null genotype for both the alleles, i.e., GSTT1/GSTM1 had significantly higher levels of serum iron (P = 0.007) and serum ferritin (P = 0.001) than patients with normal genotype for GST deletions. This is the first study to prove the role of GST gene deletions with iron overload in HbE/β thalassemia. Content Type Journal ArticleCategory Original ArticleDOI 10.1007/s00277-009-0847-yAuthors Vineeta Sharma, I.R.C.H. Building (first floor), All India Institute of Medical Sciences (AIIMS) Department of Hematology Ansari Nagar New Delhi 110 029 IndiaBijender Kumar, I.R.C.H. Building (first floor), All India Institute of Medical Sciences (AIIMS) Department of Hematology Ansari Nagar New Delhi 110 029 IndiaRenu Saxena, I.R.C.H. Building (first floor), All India Institute of Medical Sciences (AIIMS) Department of Hematology Ansari Nagar New Delhi 110 029 India Journal Annals of HematologyOnline ISSN 1432-0584Print ISSN 0939-5555

Definitions of the phenotypic manifestations of sickle cell disease

Sickle cell disease (SCD) is a pleiotropic genetic disorder of hemoglobin that has profound multiorgan effects. The low prevalence of SCD ([sim]100,000/US) has limited progress in clinical, basic, and translational research. Lack of a large, readily accessible population for clinical studies has contributed to the absence of standard definitions and diagnostic criteria for the numerous complications of SCD and inadequate understanding of SCD pathophysiology. In 2005, the Comprehensive Sickle Cell Centers initiated a project to establish consensus definitions of the most frequently occurring complications. A group of clinicians and scientists with extensive expertise in research and treatment of SCD gathered to identify and categorize the most common complications. From this group, a formal writing team was formed that further reviewed the literature, sought specialist input, and produced definitions in a standard format. This article provides an overview of the process and describes 12 body system categories and the most prevalent or severe complications within these categories. A detailed Appendix provides standardized definitions for all complications identified within each system. This report proposes use of these definitions for studies of SCD complications, so future studies can be comparably robust and treatment efficacy measured. Use of these definitions will support greater accuracy in genotype-phenotype studies, thereby achieving a better understanding of SCD pathophysiology. This should nevertheless be viewed as a dynamic rather than final document; phenotype descriptions should be reevaluated and revised periodically to provide the most current standard definitions as etiologic factors are better understood, and new diagnostic options are developed. Am. J. Hematol. 2009. © 2009 Wiley-Liss, Inc.

Erythrocytapheresis in the prevention of recurrent myocardial infarction in sickle cell disease

No abstract.

Prodromal myeloproliferative neoplasms: The 2008 WHO classification

The concept of prodromal chronic myeloproliferative neoplasms has been endorsed by the WHO classification implicating a stepwise evolution of disease. Histology of the bone marrow (BM) and borderline to mildly expressed clinical features play a pivotal role for diagnosing prefibrotic-early primary myelofibrosis. By lowering the platelet count for essential thrombocythemia and regarding BM morphology, early manifestations are tackled. Pre-polycythemic stages of polycythemia vera with a low hemoglobin level at onset are diagnosed by positive JAK2V617F mutation status, a low erythropoietin value, and characteristic BM features. The revised WHO classification incorporates hematological, morphological, and moleculargenetic parameters to generate a consensus-based working diagnosis. Am. J. Hematol., 2009. © 2009 Wiley-Liss, Inc.

Expression of CD117 by proerythroblasts

No abstract.

Unexpected immune hemolytic anemia in an intensive care ward patient

No abstract.

Recurrent acute thrombocytopenia in the hospitalized patient: Sepsis, DIC, HIT, or antibiotic-induced thrombocytopenia

No abstract.

Crystal-storing histiocytosis in plasma cell myeloma

No abstract.

Help with HELLP

No abstract.

Abnormal hematopoiesis with lysosomal vesicles and giant granules in Chediak-Higashi syndrome

No abstract.

Chronic lymphocytic leukemia cytoplasmic inclusions

No abstract.

Splenic hyalohyphomycosis, molecularly and immunologically consistent with invasive aspergillosis, in a patient with acute lymphoblastic leukemia

No abstract.

Hydroxycarbamide-induced dermopathy

No abstract.

Near total effacement of bone marrow by medulloblastoma

No abstract.

HIGH-RISK NEUROBLASTOMA: A Therapy in Evolution

ROLE OF CASPASE 8 AS A DETERMINANT IN TRAIL SENSITIVITY OF NEUROBLASTOMA CELL LINES

HYDROXYUREA-INDUCED HEMATOLOGICAL RESPONSE IN TRANSFUSION-INDEPENDENT BETA-THALASSEMIA INTERMEDIA: Case Series and Review of Literature

PERSISTENT ANTIBODY DEPLETION AFTER RITUXIMAB IN THREE CHILDREN WITH AUTOIMMUNE CYTOPENIAS

ASSOCIATION BETWEEN DEFB1 GENE HAPLOTYPE AND HERPES VIRUSES SEROPREVALENCE IN CHILDREN WITH ACUTE LYMPHOBLASTIC LEUKEMIA

HEPATOBLASTOMA METASTATIC TO THE RIGHT ATRIUM RESPONDING TO CHEMOTHERAPY ALONE

PERIPHERAL ARTERIAL TONOMETRY IN ASSESSING ENDOTHELIAL DYSFUNCTION IN PEDIATRIC SICKLE CELL DISEASE

SEVERE PURPURA FULMINANS DUE TO COEXISTENCE OF HOMOZYGOUS PROTEIN C DEFICIENCY AND HOMOZYGOUS METHYLENETETRAHYDROFOLATE REDUCTASE MUTATION

CLINICAL PROFILE AND HOME MANAGEMENT OF SICKLE CELL-RELATED PAIN: The Enugu (Nigeria) Experience

THE BENEFIT OF ATG IN IMMUNOSUPPRESSIVE THERAPY OF CHILDREN WITH MODERATE APLASTIC ANEMIA

RHABDOMYOSARCOMA OF THE EXTREMITIES: A Focus on Tumors Arising in the Hand and Foot

INCIDENCE OF PRIMARY CENTRAL NERVOUS SYSTEM TUMORS AMONG CHILDREN IN BELGRADE (SERBIA), 1991-2004

PANEL REACTIVE ANTIBODY IN THALASSEMIC SERUM INHIBITS PROLIFERATION AND DIFFERENTIATION OF CORD BLOOD CD34 + CELLS IN VITRO

ALLOGENEIC STEM CELL TRANSPLANTATION FOR MYELODYSPLASTIC SYNDROMES IN CHILDREN: A Report from the Spanish Working Party for Blood and Marrow Transplantation in Children (GETMON)

SEVERE HYPOCHROMIC MICROCYTIC ANEMIA IN A PATIENT WITH CONGENITAL ATRANSFERRINEMIA

EFFECT OF SOMATOSTATIN ANALOGUE OCTREOTIDE IN MEDULLOBLASTOMA IN XENOGRAFT AND CELL CULTURE STUDY

SUCCESSFUL TREATMENT OF KASABACH-MERRITT SYNDROME WITH VINCRISTINE AND DIAGNOSIS OF THE HEMANGIOMA USING THREE-DIMENSIONAL IMAGING

A NOVEL L218P MUTATION IN NADH-CYTOCHROME B5 REDUCTASE ASSOCIATED WITH TYPE I RECESSIVE CONGENITAL METHEMOGLOBINEMIA

PLASMA HEPARANASE AS A SIGNIFICANT MARKER OF TREATMENT RESPONSE IN CHILDREN WITH HODGKIN LYMPHOMA: Pilot Study

ALLOGENEIC CORD BLOOD TRANSPLANTATION IN CHILDREN WITH HEMATOLOGICAL MALIGNANCIES: A Long-Term Follow-Up Single-Center Study

EXTRA-OSSEOUS EWING SARCOMA

BONE MARROW TRANSPLANTATION OR HYDROXYUREA FOR SICKLE CELL ANEMIA: Long-Term Effects on Semen Variables and Hormone Profiles

ANTIBODY TITRATION AND IMMUNE RESPONSE OF IRANIAN β-THALASSEMIC PATIENTS TO HEPATITIS B VIRUSE VACCINE (BOOSTER EFFECT)

GROWTH STATUS IN CHILDREN AND ADOLESCENTS WITH SICKLE CELL DISEASE

CD10 AND CD34 EXPRESSION IN CHILDHOOD ACUTE LYMPHOBLASTIC LEUKEMIA IN MOROCCO: Clinical Relevance and Outcome

HEPATITIS G VIRUS INFECTION IN MULTITRANSFUSED EGYPTIAN CHILDREN

TRENDS IN SURVIVAL AFTER CHILDHOOD CANCER IN SLOVENIA BETWEEN 1957 AND 2007

SPERM CRYOPRESERVATION PRACTICES AMONG ADOLESCENT CANCER PATIENTS AT RISK FOR INFERTILITY

SPONTANEOUS THROMBOSIS OF HEPATIC ANEURYSMS IN AN INFANT WITH WISKOTT-ALDRICH SYNDROME

MULTIPLE CHROMOSOME ABNORMALITIES IN THE PLEURAL FLUID OF A PATIENT WITH RECURRENT EWING SARCOMA

THROMBOLYTICS FOR HYPERACUTE STROKE IN CHILDREN

HEMORHEOLOGICAL PARAMETERS IN CHILDREN WITH IRON-DEFICIENCY ANEMIA AND THE ALTERATIONS IN THESE PARAMETERS IN RESPONSE TO IRON REPLACEMENT

HAPLOIDENTICAL HEMATOPOIETIC STEM CELL TRANSPLANTATION IN CHILD HEMATOLOGIC MALIGNANCIES WITH G-CSF-MOBILIZED MARROW GRAFTS WITHOUT T-CELL DEPLETION: A Single-Center Report of 45 Cases

HYPEREOSINOPHILIC SYNDROME IN CHILDHOOD: Clinical and Molecular Features of Two Cases

THE CASE OF A 1-YEAR-OLD GIRL WITH HEREDITARY HYPERFERRITINEMIA CATARACT SYNDROME

ANAPLASTIC LARGE CELL LYMPHOMA WITH PRIMARY INVOLVEMENT OF SKELETAL MUSCLE: A Rare Case Report and Review of the Literature

POLAND SYNDROME WITH INTRACRANIAL GERM CELL TUMOR IN A CHILD

RHABDOMYOLYSIS DUE TO Escherichia coli SEPSIS IN THREE PEDIATRIC PATIENTS WITH ACUTE LYMPHOBLASTIC LEUKEMIA

PARVOVIRUS B19-INDUCED PERSISTENT PURE RED CELL APLASIA IN A CHILD WITH T-CELL IMMUNODEFICIENCY

ALTERED IRON METABOLISM IN CHILDREN WITH HUMAN IMMUNODEFICIENCY VIRUS DISEASE

ACUTE LYMPHOBLASTIC LEUKEMIA SECONDARY TO CHEMORADIOTHERAPY FOR PERIVASCULAR EPITHELIOID CELL TUMOR OF UTERUS

CHOOSING THERAPY FOR ADOLESCENT FEMALES WITH CANCER: Fertility Should Matter

RELAPSE OF PRIMITIVE MEDIASTINAL LYMPHOMA AS A MYELOID MASS FOLLOWING BEAM AUTOLOGOUS TRANSPLANT AND SUBSEQUENT REFRACTORINESS TO MATCHED UNRELATED UMBILICAL CORD BLOOD ALLOGRAFT

TRANSFORMATION OF MYELODYSPLASTIC SYNDROME TO T-CELL ACUTE LYMPHOBLASTIC LEUKEMIA IN A YOUNG ADULT

DIFFERENTIAL microRNA EXPRESSION IN CHILDHOOD B-CELL PRECURSOR ACUTE LYMPHOBLASTIC LEUKEMIA

COEXISTENCE OF SYMPTOMATIC IRON-DEFICIENCY ANEMIA AND DUODENAL NODULAR LYMPHOID HYPERPLASIA DUE TO GIARDIASIS: Case Report

EXCESSIVE NAKED MEGAKARYOCYTE NUCLEI IN MYELODYSPLASTIC SYNDROME MIMICKING IDIOPATHIC THROMBOCYTOPENIC PURPURA: A Complicated Pre- and Post-transplantation Course

SOLUBLE ENDOTHELIAL PROTEIN C RECEPTOR LEVEL IN CHILDREN WITH SEPSIS

THYROID CANCER IN LEBANESE CHILDREN AND ADOLESCENTS: A 15-Year Experience at a Single Institution

CASE STUDY: Using A Continuous Glucose Monitoring System In A Patient With Diabetes and Beta-Thalassemia Hemoglobinopathy