Higuchi T, Niikura H
Division of Hematology, Internal Medicine, Showa University Fujigaoka Hospital, Yokohama 227-8501.
Rinsho Byori. 2001 Oct;49(10):981-5.
Autoimmune hemolytic anemia (AIHA) is subclassified according to the character of the autoantibodies, however, its clinical pictures are highly heterogenous even in a given subclass. In this article, we reviewed pathogenesis, clinical and laboratory characteristics of AIHA, exemplifying data of 26 patients with autoimmune hemolysis with warm-type autoantibodies (13 AIHA, 10 Evans' syndrome, 3 systemic lupus erythematosus), and recent advances in this field. White blood cell and platelet counts varied among patients at presentation. Six of 13 AIHA patients had low reticulocyte production index (RPI) indicative of inappropriate reticulocyte response with considerable erythroblasts in the bone marrow, suggesting that ineffective erythropoiesis contributes the pathogenesis of warm-type AIHA. Ten patients were judged to require red cell transfusion. Their RPI did not differ from that of patients who did not require transfusion, indicating that the degree of hemolysis is the major determinant of the severity of the disease. No significant side effects of transfusion were observed.
自身免疫性溶血性贫血（AIHA）根据自身抗体的性质进行亚分类，然而，即使在给定的亚分类中，其临床表现也具有高度异质性。在本文中，我们回顾了AIHA的发病机制、临床和实验室特征，列举了26例伴有温型自身抗体的自身免疫性溶血患者（13例AIHA、10例Evans综合征、3例系统性红斑狼疮）的数据，以及该领域的最新进展。患者就诊时白细胞和血小板计数各不相同。13例AIHA患者中有6例网织红细胞生成指数（RPI）较低，表明网织红细胞反应不当，骨髓中有大量成红细胞，提示无效红细胞生成参与了温型AIHA的发病机制。10例患者被判定需要输注红细胞。他们的RPI与不需要输血的患者没有差异，表明溶血程度是疾病严重程度的主要决定因素。未观察到输血的明显副作用。
