Hauke G, Fauser A A, Weber S, Maas D
Blut. 1983 Jun;46(6):321-7. doi: 10.1007/BF00320692.
A patient with severe AIHA of the warm antibody type, absence of reticulocytes and red cell hyperplasia of the bone marrow is described. In order to maintain a reasonable hemoglobin level 38 units of washed packed red cells were required within 24 days. The treatment with high doses of steroids showed no permanent beneficial effect. After splenectomy the red cell destruction was immediately reduced and the patient went into a remission. Bone marrow culture studies during the acute phase of the disease and at the time of complete hemato- and immunological remission, i.e. 4 months after splenectomy suggested a circulating autoantibody directed to early erythroid progenitors (BFU-E). The inhibitory activity in the patient's plasma did not influence granulocytic or mixed colony formation (CFU-GEMM). In addition to autoantibodies directed to erythroblasts and erythropoietin involved in the pathogenic mechanisms leading to red cell aplasia type I and II the culture studies suggest an unusual autoantibody that might cause the observed reticulocytopenia and erythropoietic hyperplasia of the bone marrow in AIHA. After the splenectomy the patient recovered, he required no further blood transfusions and his disease has not recurred.
本文描述了一位患有严重温抗体型自身免疫性溶血性贫血（AIHA）的患者，该患者无网织红细胞且骨髓红细胞增生。为维持合理的血红蛋白水平，在24天内需要输注38单位的洗涤红细胞悬液。大剂量类固醇治疗未显示出持久的有益效果。脾切除术后，红细胞破坏立即减少，患者进入缓解期。在疾病急性期以及完全血液学和免疫学缓解时（即脾切除术后4个月）进行的骨髓培养研究表明，存在一种针对早期红系祖细胞（BFU-E）的循环自身抗体。患者血浆中的抑制活性不影响粒细胞或混合集落形成（CFU-GEMM）。除了针对成红细胞和促红细胞生成素的自身抗体参与导致I型和II型红细胞再生障碍的致病机制外，培养研究还提示存在一种异常自身抗体，可能导致AIHA中观察到的网织红细胞减少和骨髓红细胞生成增生。脾切除术后患者康复，无需进一步输血，且疾病未复发。
