Crow-深瀬症候群（POEMS症候群)の治療

Treatment Strategy for POEMS Syndrome

はじめに

　Crow-深瀬症候群は形質細胞腫の存在を基盤とした進行性の多発性ニューロパチーであり，そのほかに臓器腫大，内分泌異常，M蛋白血症，皮膚症状，骨硬化病変，血栓症，乳頭浮腫，腹水，胸水など多彩な症状・徴候をきたす症候群である。欧米においては主要症状の頭文字からPOEMS症候群（polyneuropathy，organomegaly，endocrinopathy，M protein，skin changes）と呼ばれる。Crow-深瀬症候群における血清VEGF（vascular endothelial growth factor）の著明な上昇が報告^1）されてからは，その病態との関連や診断および治療効果の指標としての有用性が認識されている。近年ではさまざまな治療がCrow-深瀬症候群に対して試みられ，その有効性が報告されつつある^2）。本稿ではその病態が明らかになりつつあるCrow-深瀬症候群における治療の現状を紹介する。

Abstract

　POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes) is a paraneoplastic disease associated with an underlying plasma cell dyscrasia.　This syndrome usually progresses slowly and can be life-threatening.　No large-scale controlled study providing convincing evidence for the treatment of this rare condition has thus for been conducted.　However, several lines of evidence have shown the efficacy of conventional therapies such as radiation, alkylator-based treatment, and corticosteroid therapy.　Recent studies have also proposed novel therapeutic strategies such as autologous peripheral blood stem cell transplantation (auto-PBSCT) and thalidomide, lenalidomide, and bevacizumab therapies.　We reviewed the current treatment strategies for POEMS syndrome and summarized them as follows; 1) Radiotherapy or surgical resection is highly effective for isolated plasmacytoma.　2) Melphalan-predonisolone administration may be a treatment option for the patients with widespread bone lesions.　3) High dose chemotherapy with auto-PBSCT is a promising novel approach that can lead to dramatic improvement particularly in patients with good systemic condition.　4) Plasma exchange and immunoglobulin treatment are not recommended.　5) Administration of thalidomide and lenalidomide may ameliorate the clinical conditions of the patients who are unable to undergo auto-PBSCT; however, this needs further confirmation.　Finally, we emphasized that the efficacy of these therapies largely depends on the appropriate early diagnosis of POEMS syndrome.