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POEMS syndrome is defined by the presence of a peripheral neuropathy (P), a monoclonal plasma cell disorder (M), and other paraneoplastic features, the most common of which are organomegaly (O), endocrinopathy (E), skin changes (S), papilledema, edema, effusions, ascites, and thrombocytosis. Virtually all patients will have either at least one sclerotic bone lesion or concomitant Castleman disease. The peak incidence of the POEMS syndrome is in the 5th and 6th decades of life. The prevalence of POEMS syndrome is unknown. Not all features of the disease need to be present to make the diagnosis, and early recognition is important to reduce morbidity. POEMS syndrome is frequently confused with chronic inflammatory demyelinating polyneuropathy. This misdiagnosis is problematic since therapies that are effective in patients with chronic inflammatory demyelinating polyneuropathy (intravenous gammaglobulin, plasmapheresis, and azathioprine) are not effective in patients with POEMS. The cause of POEMS syndrome is unknown. The mainstays of therapy for patients with POEMS include irradiation, corticosteroids, and alkylator-based therapy, including high dose chemotherapy with peripheral blood stem cell transplant. |