Department of Pediatrics, Matsushita Memorial Hospital, Moriguchi, Japan.
J Hematol Oncol. 2009 Sep 10;2:40. doi: 10.1186/1756-8722-2-40.
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.
遗传性球形红细胞增多症（HS）是一种红细胞膜的遗传性疾病，临床上表现为贫血、黄疸和脾肿大。Evans 综合征是一种临床综合征，其特征是自身免疫性溶血性贫血（AIHA）伴免疫性血小板减少性紫癜（ITP）。它是由免疫系统功能障碍引起的，产生针对至少红细胞和血小板的多种自身抗体。HS 和 Evans 综合征的病理生理学机制彼此不同。我们报告了一例同时患有这两种疾病的婴儿病例。讨论了这种意外并发症的可能解释。
