POEMS syndrome

Joan Blade MD PhD (

Dr. Blade of Hospital Clinic of Barcelona received honorariums for educational lectures and consulting fees from Janssen, Celgene, Amgen, and Takeda.

)
M Teresa Cibeira MD PhD (

Dr. Cibeira of Hospital Clinic of Barcelona received honorariums for educational lectures from Janssen, Celgene, and Amgen and consulting fees from Janssen.

)
Francesc Graus MD PhD, editor. (Dr. Graus of the University of Barcelona has no relevant financial relationships to disclose.)
Originally released May 8, 1995; last updated January 24, 2019; expires January 24, 2022

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

Overview

POEMS syndrome is a rare multisystem disease with unknown pathogenesis; it is classified as a plasma cell dyscrasia. Although neuropathy is the dominant clinical feature, the syndrome is characterized by a constellation of manifestations, including but not limited to those referred by the acronym: polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, and skin changes. In this article, the authors discuss the biological basis, clinical presentation, diagnosis, and treatment options, including autologous peripheral blood stem cell transplantation and other emerging therapies.

Key points

 

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

 

• The acronym POEMS refers to polyneuropathy (dominant clinical feature), 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.

 

• The aim of therapy is to target the underlying plasma cell clone with a risk-adapted therapy.

Historical note and terminology

The first case of what we now know as POEMS syndrome was reported in 1938 by Scheinker (Scheinker 1938). Almost 20 years later, Crow described 2 patients with multiple myeloma and peripheral neuropathy in his paper titled “Peripheral neuritis in myelomatosis.” Both patients presented with peripheral neuropathy and had lytic and sclerotic bone lesions, skin pigmentation, and normal sedimentation rates (Crow 1956). Twelve years later, other author 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 and postulated this to be a new syndrome (Yodoi et al 1973). A few years later, Takatsuki and coworkers 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 term POEMS (polyneuropathy, organomegaly, endocrinopathy, presence of a monoclonal band and skin changes) (Bardwick et al 1980). It has also been referred as Crow-Fukase syndrome, Takatsuki syndrome, PEP syndrome (plasma cell dyscrasia, endocrine disturbances and polyneuropathy), osteosclerotic myeloma, and Japanese multisystem syndrome.

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