POEMS syndrome

Jai Grewal MD (Dr. Grewal of the Long Island Brain Tumor Center at Neurological Surgery, PC, received research funding from Northwest Biotherapeutics and consulting fees from Novocure.)
Harpreet K Grewal MD (Dr. Grewal of JGMDPC in Roslyn, New York, has no relevant financial relationships to disclose; Dr. Grewal's spouse received research funding from Northwest Biotherapeutics and consulting fees from Novocure.)
Raymond P Roos MD, editor. (Dr. Roos of the University of Chicago owns stock in Amgen, Express Scripts, Isis, and Merck.)
Originally released May 8, 1995; last updated April 25, 2014; expires April 25, 2017

This article includes discussion of POEMS syndrome, Crow-Fukase syndrome, Japanese multisystem disease, plasma cell dyscrasia, PEP syndrome, and Takatsuki syndrome. The foregoing terms may include synonyms, similar disorders, variations in usage, and abbreviations.

Overview

POEMS syndrome is a multisystem disease that also features various other systemic manifestations such as sclerotic bone lesions, Castleman disease, peripheral edema, and thrombocytosis. The pathogenesis is unknown, but the symptoms are likely secondary to the clonal plasmaproliferative disorder. In this article, the authors describe emerging treatment strategies for POEMS syndrome, including autologous peripheral blood stem cell transplantation and other new therapies.

Key points

 

• POEMS syndrome is a rare, incompletely understood multisystem disease caused by an underlying plasma cell dyscrasia.

 

• The acronym POEMS refers to polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes, although many other features may be intermixed.

 

• The differential diagnosis includes other causes of polyneuropathy, skeletal abnormality, and paraproteinemia, including diabetes, porphyria, heavy metal toxicity, other plasma cell dyscrasia-related polyneuropathies, sarcoidosis, chronic inflammatory demyelinating neuropathies, paraneoplastic disorders, and hereditary demyelinating states.

 

• There is no consensus regarding a standard therapeutic regimen for POEMS syndrome, and there are no randomized controlled trials (Kuwabara et al 2008b). Most of the recommendations for treatment have been derived from small numbers of cases.

Historical note and terminology

A paper titled “Peripheral neuritis in myelomatosis” appeared in the British Medical Journal in 1956. The author, RS Crow, was a medical senior registrar. He described 2 patients with multiple myeloma and peripheral neuropathy. Both patients presented with peripheral neuropathy, had lytic and sclerotic bone lesions, skin pigmentation, and normal sedimentation rates (Crow 1956). In 1968 Shimpo described a patient with plasmacytoma, polyneuritis, and endocrine abnormalities (Shimpo et al 1968). In 1973 Yodoi described the association of myeloma, polyneuropathy, endocrinopathy, and skin pigmentation. He then postulated this to be a new syndrome (Yodoi et al 1973). Takatsuki later reviewed 32 patients with polyneuropathy, endocrinopathy, and a plasma cell disorder (Takatsuki et al 1977). In 1980 Bardwick and colleagues described similar findings in American patients and coined the syndrome POEMS (polyneuropathy, organomegaly, endocrinopathy, presence of a monoclonal band and skin changes) (Bardwick et al 1980). Its various eponyms are Crow-Fukase syndrome, Takatsuki syndrome, plasma cell dyscrasia, endocrine disturbances, polyneuropathy syndrome (also called PEP syndrome), and Japanese multisystem syndrome.

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