免疫性溶血性贫血

A. Taksande, Snehi Kumar
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引用次数: 0

摘要

自身免疫性溶血性贫血(AIHA)的诊断对免疫血液学实验室和临床医生都是一个挑战,因为实验室调查可能很麻烦,通常需要大量耗时的血清学检测,特别是在需要输血时。通常,需要迅速开始治疗。针对红细胞表位的自身抗体在AIHA的发病机制中起着至关重要的作用,这些表位包含糖和/或蛋白质结构。同型对自身抗体的临床意义十分重要。通常,患者有黄疸和贫血的临床症状,如苍白、疲劳、呼吸急促和心悸,检查时可出现肝脾肿大。如果没有需要输血的重要指征,则应谨慎等待免疫血液学检查的结果和随后基于此的输血建议。治疗的重点应该是停止溶血,或至少通过抑制自身抗体的产生和/或抑制过早的红细胞破坏来减轻。只有当基础疾病得到治疗时,继发性AIHA才有可能成功治疗。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Immune Hemolytic Anemia
The diagnosis of autoimmune haemolytic anaemia (AIHA) is a challenge for both the immune-haematology laboratory and the clinician as the laboratory investigation can be troublesome and often requires extensive time-consuming serological testing, especially when a blood transfusion is needed. Frequently, there is a need to start therapy rapidly. Autoantibodies directed to epitopes on red blood cell (RBC) consisting in sugar and/or protein structures are crucial in the pathogenesis of AIHA. The isotype is important for the clinical significance of an autoantibody. Frequently, patients are icteric and suffer from clinical signs of anaemia, such as pallor, fatigue, shortness of breath and palpitations, on examination hepatosplenomegaly can be present. If there is no vital indication for a transfusion it is prudent to wait for the results of the immune-haematological tests and the ensuing transfusion advice based on this. Focus of treatment should be stoppage of hemolysis or at least be attenuated via an inhibition of autoantibody production and/or inhibition of premature RBC destruction. Successful treatment of secondary AIHA is only possible when the underlying disease is treated.
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