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[与特发性混合型自身免疫性溶血性贫血相关的伊文氏综合征]

[Evans syndrome associated with idiopathic mixed-type autoimmune hemolytic anemia].

作者信息

Kakinoki Yasutaka, Nagase Masako, Okada Kohei, Chiba Koji, Fukuhara Takashi, Miyake Takayoshi

机构信息

Division of Hematology/Oncology, Department of Internal Medicine, Asahikawa City Hospital.

出版信息

Rinsho Ketsueki. 2008 Jul;49(7):516-20.

Abstract

A 60-year-old man was admitted to our hospital with severe anemia and blood findings showed hemolytic anemia. Further serological examination revealed both warm-reactive autoantibody and cold agglutinin against erythrocytes. The cold agglutinin showed a low titer, 1 : 32 at 4 degrees C, and had a high thermal amplitude of 30 degrees C or higher, resulting in sufficient activity for hemolysis. Since no underlying disorders could be detected, the diagnosis was idiopathic mixed-type autoimmune hemolytic anemia. Although thrombocytopenia (Evans syndrome) subsequently appeared, corticosteroid was extremely effective for both anemia and thrombocytopenia. In this report we describe a rare case of Evans syndrome associated with mixed-type autoimmune hemolytic anemia, which had a dramatic response to corticosteroid therapy.

摘要

一名60岁男性因严重贫血入院,血液检查显示为溶血性贫血。进一步的血清学检查发现了针对红细胞的温反应性自身抗体和冷凝集素。冷凝集素滴度较低,4℃时为1:32,热振幅较高,达30℃或更高,具有足够的溶血活性。由于未发现潜在疾病,诊断为特发性混合型自身免疫性溶血性贫血。尽管随后出现了血小板减少症(伊文氏综合征),但皮质类固醇对贫血和血小板减少症都极为有效。在本报告中,我们描述了一例罕见的与混合型自身免疫性溶血性贫血相关的伊文氏综合征病例,该病例对皮质类固醇治疗有显著反应。

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