摘要

Hereditary spherocytosis (HS) is a genetic disorder of the red blood cell membrane clinically characterized by anemia, jaundice and splenomegaly. Evans' syndrome is a clinical syndrome characterized by autoimmune hemolytic anemia (AIHA) accompanied by immune thrombocytopenic purpura (ITP). It results from a malfunction of the immune system that produces multiple autoantibodies targeting at least red blood cells and platelets. HS and Evans' syndrome have different mechanisms of pathophysiology one another. We reported the quite rare case of an infant who had these diseases concurrently. Possible explanations of the unexpected complication are discussed.

译文

遗传性球形细胞增多症 (HS) 是一种以贫血、黄疸和脾肿大为临床特征的红细胞膜遗传性疾病。Evans 综合征是一种以自身免疫性溶血性贫血 (AIHA) 伴免疫性血小板减少性紫癜 (ITP) 为特征的临床综合征。它是由免疫系统故障引起的，免疫系统产生了多种针对至少红细胞和血小板的自身抗体。HS 和 evans 综合征具有不同的病理生理机制。我们报告了一个相当罕见的同时患有这些疾病的婴儿病例。讨论了意外并发症的可能解释。