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多中心Castleman病合并POEMS综合征患者造血干细胞移植成功。

Successful hematopoietic stem-cell transplantation in multicentric Castleman disease complicated by POEMS syndrome.

作者信息

Ganti Apar Kishor, Pipinos Iraklis, Culcea Eliad, Armitage James O, Tarantolo Stefano

机构信息

Department of Internal Medicine, Division of Oncology/Hematology, University of Nebraska Medical Center, Omaha, Nebraska 68198-7680, USA.

出版信息

Am J Hematol. 2005 Jul;79(3):206-10. doi: 10.1002/ajh.20280.

Abstract

A 39-year-old male presented with pedal edema, pleural effusion, splenomegaly, and generalized lymphadenopathy. Serum protein electrophoresis demonstrated the presence of a monoclonal protein. Histological examination of the spleen following splenectomy showed multifocal vascular proliferation and angiovascular lesions consistent with multicentric Castleman disease. He was treated with steroids and rituximab, but without improvement. The patient was found to have portal venous thrombosis and lower extremity arterial thrombosis. He then received combination chemotherapy with cyclophosphamide and mitoxantrone but developed a severe inflammatory polyneuropathy that left him disabled and wheelchair-bound. A diagnosis of multicentric Castleman disease with POEMS syndrome was made, and he then received high-dose chemotherapy with melphalan followed by autologous peripheral blood stem-cell transplantation. Following transplantation, his nerve conduction studies improved and his serum protein electrophoresis normalized. He is currently ambulatory and does not need wheelchair assistance. Hematopoietic stem-cell transplantation may be a treatment option for patients with multicentric Castleman disease and POEMS syndrome.

摘要

一名39岁男性出现足部水肿、胸腔积液、脾肿大和全身淋巴结病。血清蛋白电泳显示存在单克隆蛋白。脾切除术后脾脏的组织学检查显示多灶性血管增生和心血管病变,符合多中心Castleman病。他接受了类固醇和利妥昔单抗治疗,但无改善。该患者被发现患有门静脉血栓形成和下肢动脉血栓形成。随后他接受了环磷酰胺和米托蒽醌联合化疗,但出现了严重的炎性多神经病,导致他残疾并需依赖轮椅。诊断为多中心Castleman病合并POEMS综合征,随后他接受了马法兰大剂量化疗,接着进行了自体外周血干细胞移植。移植后,他的神经传导研究结果改善,血清蛋白电泳恢复正常。他目前能够行走,无需轮椅辅助。造血干细胞移植可能是多中心Castleman病和POEMS综合征患者的一种治疗选择。

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