POEMS syndrome

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).

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Diagnosis

It was originally described in part by various investigators, and received its present name in 1980 by Bardwick et al^[1].

A 2003 study^[2] 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 criterion 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

In addition, their study showed a number of associations, the relevance of which to diagnosis was unclear: clubbing, weight loss, thrombocytosis, polycythemia and hyperhidrosis. Possible associations included pulmonary hypertension, restrictive lung disease, a thrombotic diathesis, arthralgia, cardiomyopathy (systolic dysfunction), fever, low vitamin B12 levels and diarrhea.

Pathogenesis

While the main features of the disease have been described, the exact mechanism of the disease has remained elusive. The paraprotein alone is insufficient to explain the multi-organ features, and various cytokines produced by plasmacytoma cells have therefore been linked with the features of POEMS syndrome, specifically interleukin 1β, interleukin 6 and TNFα. There also seems to be a role of vascular endothelial growth factor, given the angiogenetic changes in many tissues.^[2]

Treatment

Dispenzieri et al^[2] list numerous treatments, the effect of many of which is almost anecdotal. Prednisolone and alkylating agents are the most commonly used.

References

1. ^ Bardwick 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.
2. ^ ^{a b c} Dispenzieri 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.