How I treat POEMS syndrome.
Blood. 2012 Apr 30;
Authors: Dispenzieri A
POEMS syndrome is a paraneoplastic syndrome whose acronym stands for less than half of the defining features of the disease, that is polyradiculoneuropathy, organomegaly, potentially including co-existing Castleman's disease, endocrinopathy, monoclonal plasma cell neoplasm, and skin changes. The other important features include papilledema, extravascular volume overload, sclerotic bone lesions, thrombocytosis, elevated VEGF, and abnormal pulmonary function. The diagnosis is based on having both the polyradiculoneuropathy and the monoclonal plasma cell disorder, and at least one of the other 3 major criteria (Castleman's disease, sclerotic bone lesions, or elevated VEGF) and at least one minor criterion. The diagnosis is often delayed with intervening incorrect diagnoses of chronic inflammatory demyelinating polyradiculoneuropathy, myeloproliferative disorder, and monoclonal gammopathy of undetermined significance. Prompt treatment directed at the underlying plasma cell clone produces dramatic responses in the majority of patients. Although there are no randomized clinical trial data to direct best therapy, for patients with disseminated disease high dose chemotherapy with peripheral blood transplantation has yielded durable benefit, while radiation therapy is typically effective for patients with a more localized presentation. More universal recognition of and more scientific inquiry into the underpinnings of the disease will provide direction towards the best treatment strategies in the future.
PMID: 22547581 [PubMed - as supplied by publisher]