Crow-Fukase syndrome, also called POEMS syndrome, is a rare paraneoplastic syndrome due to an underlying plasma cell neoplasm, characterized by polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes as its salient features. Major diagnostic criteria include sclerotic bone lesions, elevated serum or plasma levels of vascular endothelial growth factor (VEGF), and Castleman disease. The misdiagnosis is very common due to its rarity and complicated clinical manifestations. Recognition of the complex of clinical complications is the first step in effectively managing the disease. The pathogenesis is not well understood. VEGF appears to play an important role in the disease and is especially useful for monitoring therapy, but it is not likely the sole factor driving the disease. The most commonly used therapies include alkylators and steroids, high-dose chemotherapy with peripheral blood stem cell transplantation, lenalidomide, and bortezomib.
ICD-11 による疾患分類 [BR:
特定疾患 (難病) [
Dispenzieri A, Buadi FK
A review of POEMS syndrome.
Oncology (Williston Park) 27:1242-50 (2013)
Cui RT, Yu SY, Huang XS, Zhang JT, Tian CL, Dou LP, Pu CQ
Incidence and risk factors of pleural effusions in patients with POEMS syndrome.
Hematol Oncol 33:80-4 (2015)
Raheja D, Specht C, Simmons Z
Muscle Nerve 51:1-13 (2015)
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