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一名血友病患者的获得性免疫缺陷综合征

Acquired immunodeficiency syndrome in a patient with hemophilia.

作者信息

Davis K C, Horsburgh C R, Hasiba U, Schocket A L, Kirkpatrick C H

出版信息

Ann Intern Med. 1983 Mar;98(3):284-6. doi: 10.7326/0003-4819-98-3-284.

Abstract

A patient with hemophilia A developed T-cell deficiency characterized by infection with several opportunistic pathogens. Immunologic investigation showed cutaneous anergy, lymphocyte unresponsiveness to mitogens and antigens, an abnormal ratio of T-helper and T-suppressor cells with absolute lymphopenia and elevated IgA. The clinical and immunologic characteristics of this patient fit the recently described syndrome of opportunistic infections or Kaposi's sarcoma in patients with acquired T-cell deficiency; however, this patient does not have any of the associated underlying risk factors such as homosexuality, intravenous drug or amyl nitrite use, or positive serologic tests for syphilis. We conclude that the patient's acquired T-cell deficiency can be explained by exposure to a virus or other transmissible agent during factor VIII transfusions.

摘要

一名甲型血友病患者出现了以感染多种机会性病原体为特征的T细胞缺陷。免疫学调查显示皮肤无反应性、淋巴细胞对有丝分裂原和抗原无反应、辅助性T细胞和抑制性T细胞比例异常,伴有绝对淋巴细胞减少和IgA升高。该患者的临床和免疫学特征符合最近描述的获得性T细胞缺陷患者的机会性感染或卡波西肉瘤综合征;然而,该患者没有任何相关的潜在危险因素,如同性恋、静脉注射毒品或使用亚硝酸戊酯,或梅毒血清学检测呈阳性。我们得出结论,该患者获得性T细胞缺陷可归因于在输注凝血因子VIII期间接触了病毒或其他可传播因子。

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