POEMS syndrome: Diagnosis, treatment and the current status in Japan

AbstractPolyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome is a rare cause of demyelinating polyneuropathy associated with monoclonal proliferation of plasma cells. Because the disorder shows diverse symptoms, its diagnosis is often difficult. Previously reported diagnostic criteria had many items and their accuracy was not confirmed. Recently new diagnostic criteria are proposed, which consist of only seven items and have better diagnostic accuracy.Since around 2000, treatments for myeloma, such as high ‐dose chemotherapy with autologous stem cell transplantation, immunomodulatory drugs, and proteasome inhibitors, have been applied to POEMS syndrome. A recent national survey conducted in Japan has revealed that the prognosis has been prominently improved by these plasma cell‐targeting therapeut ic interventions: 10‐year overall survival was 93%. Conversely, 24% of the patients were still treated with corticosteroid alone as an initial treatment, suggesting that recognition of the disorder is not sufficient.Accurate diagnosis and proper treatment in the early phase of the disease are essential to further improve prognosis of POEMS syndrome. The present review focuses on diagnosis, treatment, and the current status of the disease in Japan.
Source: Clinical and Experimental Neuroimmunology - Category: Neurology Authors: Tags: REVIEW ARTICLE (INVITED) Source Type: research