Polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes syndrome: Diagnosis, treatment and the current status in Japan

Patients with polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes syndrome are currently treated with therapeutic options for multiple myeloma, and this disease is a treatable disease. Accurate diagnosis and proper treatment in the early stage of the disease are essential to further improve prognosis. This review focuses on diagnosis, treatment and the current status of polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes syndrome 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 have been proposed, which consist of just 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 polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes syndrome. A recent national survey carried out in Japan showed that the prognosi s has been prominently improved by these plasma cell‐targeting therapeutic interventions: 10‐year overall survival ...
Source: Clinical and Experimental Neuroimmunology - Category: Neurology Authors: Tags: REVIEW ARTICLE Source Type: research