INTRODUCTION

POEMS syndrome is a paraneoplastic disorder associated with an underlying plasma cell dyscrasia. The major clinical feature of the syndrome is a chronic progressive polyneuropathy with a predominant motor disability. The acronym POEMS (Polyneuropathy, Organomegaly, Endocrinopathy, M protein, and Skin changes) captures several dominant features of the syndrome. Important traits not included in the acronym include elevated levels of vascular endothelial growth factor (VEGF), sclerotic bone lesions, Castleman disease, papilledema, peripheral edema, ascites, effusions, thrombocytosis, polycythemia, fatigue, clubbing, and abnormal pulmonary function test. Other names for the syndrome include osteosclerotic myeloma, Crow-Fukase syndrome, and Takatsuki syndrome. Although the vast majority of patients have osteosclerotic myeloma, these same patients usually have only 5% bone marrow plasma cells or less (almost always monoclonal λ), and rarely have anemia, hypercalcemia, or renal insufficiency. These characteristics and the superior median survival differentiate POEMS syndrome from multiple myeloma.

The complexity of the interaction of plasma cell dyscrasia and peripheral neuropathy (PN) became increasingly evident in 1956 with Crow's description of two patients with osteosclerotic plasmacytomas with neuropathy, and other “striking features,” which included clubbing, skin pigmentation, dusky discoloration of skin, white finger nails, mild lymphadenopathy, and ankle edema. As many as 50% of patients with osteosclerotic myeloma were noted to have PN in contrast to 1–8 of multiple myeloma patients. A syndrome distinct from multiple myeloma-associated neuropathy became to be recognized.