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. (Clin. Exp. Neuroimmunol.

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

“…The diagnosis of POEMS syndrome is made based on the diagnostic criteria, which consist of characteristic clinical features and laboratory abnormalities . Dispenzieri’s criteria have been used internationally; in contrast, in Japan, modified criteria proposed by Misawa and Kuwabara have been used .…”

“…The diagnosis of POEMS syndrome is made based on the diagnostic criteria, which consist of characteristic clinical features and laboratory abnormalities . Dispenzieri’s criteria have been used internationally; in contrast, in Japan, modified criteria proposed by Misawa and Kuwabara have been used . As the symptoms of POEMS syndrome are diverse, every clinical and laboratory finding should be checked based on the criteria; however, making the diagnosis of POEMS syndrome includes several issues …”

“…Since the 2000s, treatments for multiple myeloma, such as high‐dose chemotherapy (HDT) with autologous stem cell transplantation (ASCT), immunomodulatory drugs and proteasome inhibitors, have been applied to POEMS and considered to improve its prognosis dramatically . In contrast, some patients might not recover sufficiently, mainly due to delayed diagnosis, which results in poor outcomes …”

POEMS neuropathy: optimising diagnosis and management

“…Patients with POEMS with Castleman’s disease appear to have a less severe neuropathy, which may explain why there can be such heterogeneity. There are other cases of atypical POEMS in the literature with an argument to redefine the diagnostic criteria and include the entity of Castleman’s disease variant of POEMS 24. An atypical presentation of POEMS with a positive VEGF is probably more likely to be POEMS than another disease, and therefore VEGF is a useful test when faced with an atypical presentation.…”

Proposal of new clinical diagnostic criteria for POEMS syndrome

“…Since around the 2000, therapeutic interventions available for multiple myeloma, such as autologous peripheral blood stem cell transplantation, thalidomide/lenalidomide and proteasome inhibitor therapies, have been utilised in patients with POEMS syndrome, which have improved its prognosis substantially 5 6. However, many patients do not improve sufficiently largely because of delayed diagnosis, which results in poor outcomes 7. Diagnosis of POEMS syndrome in the early disease stage is often challenging, particularly in patients who have polyneuropathy as an isolated symptom.…”