Polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes (Crow-Fukase) syndrome: Diagnostic criteria and treatment perspectives

Abstract

Polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes (POEMS; Crow-Fukase) syndrome is a devastating disorder related to plasma cell dyscrasia, characterized by polyneuropathy, organomegaly, edema/effusion, M-protein and skin changes. Novel therapeutic approaches can substantially improve the prognosis. Diagnosis of POEMS syndrome is often challenging because of a combination of clinical presentations. To facilitate precise and early diagnosis, a comprehensive systemic survey is essential, and we propose that current diagnostic criteria should be modified. The current first-line therapy is high-dose chemotherapy with autologous stem cell transplantation, whereas patients who are not eligible for transplantation because of older age or poor general condition, are treated with immunomodulatory drugs, such as thalidomide or lenalidomide. The prognosis of POEMS syndrome has been substantially improved by these novel interventions. However, how to treat very young patients and those with a relapse after autologous stem cell transplantation is controversial. Until the 2000s, therapeutic approaches initially aimed at lifesaving (or short-term improvement), but recent treatment advances could achieve long-term remission of the disease. A treatment algorithm should be decided from the viewpoint of disease control over a decade. Elucidation of the natural history of the disease and well-conducted randomized control studies are fundamental to establish a proper therapeutic guideline. The present review focuses on recent advances in the diagnosis and treatment of POEMS syndrome, and discusses future perspectives of therapeutic strategies. © 2013 Japanese Society for Neuroimmunology.