POEMS syndrome (also known as Crow-Fukase syndrome, Takatsuki disease, or PEP syndrome) is a rare medical syndrome named for its main clinically recognizable features: Polyneuropathy (peripheral nerve damage), Organomegaly (abnormal enlargement of organs), Endocrinopathy (damage to hormone-producing glands)/Edema, M-protein (an abnormal antibody) and Skin abnormalities (including hyperpigmentation and hypertrichosis).

Diagnosis

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It was originally described in part by various investigators, and received its present name in 1980 by Bardwick et alBardwick PA, Zvaifler NJ, Gill GN, Newman D, Greenway GD, Resnick DL. Plasma cell dyscrasia with polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes: the POEMS syndrome: report on two cases and a review of the literature. Medicine (Baltimore) 1980;59:311-22. PMID 6248720..

A 2003 studyDispenzieri A, Kyle RA, Lacy MQ, Rajkumar SV, Therneau TM, Larson DR, Greipp PR, Witzig TE, Basu R, Suarez GA, Fonseca R, Lust JA, Gertz MA. POEMS syndrome: definitions and long-term outcome. Blood 2003;101:2496-506. PMID 12456500. followed a series of 99 patients and attempted to delineate criteria for the diagnosis of POEMS syndrome. According to their study, two major criteria and one minor criterium are required for the diagnosis:

• Major criteria:
  • Polyneuropathy
  • Monoclonal plasmaproliferative disorder (a paraprotein)
• Minor criteria:
  • Sclerotic bone lesions
  • Castleman disease
  • Organomegaly (splenomegaly, hepatomegaly or lymphadenopathy)
  • Edema (edema, pleural effusion or ascites)
  • Endocrinopathy (adrenal, thyroid, pituitary, gonadal, parathyroid, pancreatic)
  • Skin changes (hyperpigmentation, hypertrichosis, plethora, hemangiomata, white nails)
  • Papilledema

More on [ POEMS syndrome ]

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