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[伴有28年病史的多中心Castleman病相关自身免疫性溶血性贫血]

[Autoimmune hemolytic anemia associated with multicentric Castleman's disease with a 28-year history].

作者信息

Hisatake J, Ishiyama T, Akimoto Y, Matsuda I, Hino K, Tomoyasu S, Tsuruoka N, Ota H, Kazama K

机构信息

Department of Hematology, Showa University.

出版信息

Rinsho Ketsueki. 1994 Aug;35(8):768-73.

PMID:7933564
Abstract

A 49-year-old female admitted because of anemia. had skin rashes since age 20. Generalized lymphadenopathy and fever appeared and the patient was diagnosed as multicentric Castleman's disease (MCD) at 40 years of age. Lymphadenopathy and fever improved with combined chemotherapy. In November, 1992, anemia increased with reticulocytosis (11.8%) and laboratory examination revealed a positive result for Coombs test and increased indirect bilirubin. A diagnosis of autoimmune hemolytic anemia (AIHA) was made. Steroid and plasmapheresis showed temporary effects, but anemia relapsed when steroids were decreased. Immunosuppressive drugs, vincristine and danazole were ineffective. Anemia improved on the second attempt at steroid therapy. The level of Hb rose to 11.2 g/dl after 3 months. The relationship between MCD and AIHA was discussed.

摘要

一名49岁女性因贫血入院。自20岁起出现皮疹。40岁时出现全身淋巴结肿大和发热,患者被诊断为多中心Castleman病(MCD)。联合化疗后淋巴结肿大和发热有所改善。1992年11月,贫血加重,网织红细胞增多(11.8%),实验室检查显示库姆斯试验阳性,间接胆红素升高。诊断为自身免疫性溶血性贫血(AIHA)。类固醇和血浆置换显示有暂时效果,但类固醇减量时贫血复发。免疫抑制药物、长春新碱和达那唑无效。第二次类固醇治疗尝试时贫血有所改善。3个月后血红蛋白水平升至11.2 g/dl。讨论了MCD与AIHA之间的关系。

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