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一名患有严重自身免疫性溶血性贫血的女婴:明显的抗-Vel特异性。

An infant girl with severe autoimmune hemolytic anemia: apparent anti-Vel specificity.

作者信息

Becton D L, Kinney T R

出版信息

Vox Sang. 1986;51(2):108-11. doi: 10.1111/j.1423-0410.1986.tb00224.x.

DOI:10.1111/j.1423-0410.1986.tb00224.x
PMID:3776136
Abstract

Autoantibodies directed against the common red-cell antigen Vel are unusual, with only 2 previous cases reported. This report describes an infant girl with steroid-resistant autoimmune hemolytic anemia whose serum contained an antibody with apparent specificity for Vel antigen. Transfusion with Vel-positive cells resulted in a massive hemolytic transfusion reaction. Survival of labelled Vel-negative cells was normal up to 8 h following transfusion, and no transfusion reaction followed subsequent use of Vel-negative cells. There was no evidence of a collagen vascular disease, an immunodeficiency syndrome, or a malignancy. Although rare, anti-Vel should be considered in unexplained autoimmune hemolytic anemia.

摘要

针对常见红细胞抗原Vel的自身抗体并不常见,此前仅报告过2例。本报告描述了一名患有类固醇抵抗性自身免疫性溶血性贫血的女婴,其血清中含有一种对Vel抗原有明显特异性的抗体。输注Vel阳性细胞导致了严重的溶血性输血反应。输注后8小时内,标记的Vel阴性细胞存活正常,随后使用Vel阴性细胞未发生输血反应。没有证据表明存在胶原血管疾病、免疫缺陷综合征或恶性肿瘤。尽管罕见,但在不明原因的自身免疫性溶血性贫血中应考虑抗Vel抗体。

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