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.
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.
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• POEMS syndrome is a rare multisystem disease caused by an underlying plasma cell dyscrasia.
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• The acronym POEMS refers to polyneuropathy (dominant clinical feature), organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes, although many other features may be intermixed.
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• 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.
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• 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 (79). 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 (15). Twelve years later, other author described a patient with plasmacytoma, polyneuritis, and endocrine abnormalities (84). In 1973, Yodoi described the association of myeloma, polyneuropathy, endocrinopathy, and skin pigmentation and postulated this to be a new syndrome (102). A few years later, Takatsuki and coworkers reviewed 32 patients with polyneuropathy, endocrinopathy, and a plasma cell disorder (91). 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) (06). 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.