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脾切除术使一名患有多中心Castleman病和自身免疫性溶血性贫血的患者完全缓解。

Splenectomy induced complete remission in a patient with multicentric Castleman's disease and autoimmune hemolytic anemia.

作者信息

Lerza R, Castello G, Truini M, Ballarino P, Tredici S, Cavallini D, Pannacciulli I

机构信息

Cattedra di Clinica Medica R., Dipartimento di Medicina Interna, Università di Genova, Italy.

出版信息

Ann Hematol. 1999 Apr;78(4):193-6. doi: 10.1007/s002770050500.

Abstract

Castleman's disease (CD) is a rare disorder of the lymphoid tissue in which the clinical manifestations often mimic a malignant lymphoma. Despite the absence of monoclonality of the lymphoid proliferation, the multicentric variant of the disease (MCD) is characterized by severe symptoms and poor prognosis. Etiologic, pathogenetic, and therapeutic aspects of MCD are still uncertain. We report the case of a 57-year-old patient affected by MCD complicated by severe immunohemolytic anemia. Whereas the clinical and laboratory response to steroids and chemotherapeutic agents was only partial, splenectomy induced a complete remission of hemolysis and disappearance of the constitutional symptoms and of all generalized lymphadenopathies.

摘要

卡斯特曼病(CD)是一种罕见的淋巴组织疾病,其临床表现常类似恶性淋巴瘤。尽管淋巴组织增生缺乏单克隆性,但该疾病的多中心型(MCD)具有症状严重、预后不良的特点。MCD的病因、发病机制及治疗方面仍不明确。我们报告一例57岁患有MCD并伴有严重免疫性溶血性贫血的患者。虽然对类固醇和化疗药物的临床及实验室反应仅为部分有效,但脾切除术使溶血完全缓解,全身症状及所有全身淋巴结肿大消失。

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