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

Abstract

Polyneuropathy, 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 prognosis has been prominently improved by these plasma cell‐targeting therapeutic 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 the prognosis of polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes syndrome. The present review focuses on diagnosis, treatment and the current status of the disease in Japan.

Detection of clonal plasma cells in POEMS syndrome using multiparameter flow cytometry

“…POEMS syndrome is a chronic, progressive disorder with a poor prognosis. However, POEMS syndrome prognosis significantly improves with early diagnosis due to increased awareness of the disease, aggressive indications for autologous hematopoietic cell transplantation following high-dose melphalan, and the introduction of several novel agents similar to those used for treating multiple myeloma (MM), such as bortezomib-based or lenalidomide-based regimens 4 , 5 .…”