Chaplin H, Zarkowsky H S
Arch Intern Med. 1981 Jul;141(8):1091-3.
Four patients experienced combined sickle cell disease and autoimmune hemolytic anemia within the past ten years. A fifth patient had positive direct antiglobulin test results without verified autoimmune hemolysis. Severely accelerated hemolysis was observed in four patients; anemia was severe, and the reticulocyte count rose into the 60% to 88% range. During the period of active autoimmune hemolysis, decline of the reticulocyte count rose into the 60% to 88% range. During the period of active autoimmune hemolysis, decline of the reticulocyte count into the 6% to 16% range was associated with rapid decrease in the hemoglobin level, requiring transfusion. All five patients were already alloimmunized by transfusions administered before onset of the autoimmune hemolytic anemia; two or more allospecificities were identified in four of five patients. The presence of autoantibody notably compromised compatibility testing; three patients experienced posttransfusion hemoglobinuria, and in vivo cross matching with 51Cr-labeled donor RBCs was employed on three occasions. All patients responded to corticosteroids; mercaptopurine was also administered to one patient. The direct antiglobulin test result reverted to negative in all patients after hospital discharge.
在过去十年中，有四名患者同时患有镰状细胞病和自身免疫性溶血性贫血。第五名患者直接抗球蛋白试验结果呈阳性，但未证实存在自身免疫性溶血。四名患者出现严重加速溶血；贫血严重，网织红细胞计数升至60%至88%。在自身免疫性溶血活跃期，网织红细胞计数降至6%至16%与血红蛋白水平迅速下降有关，需要输血。所有五名患者在自身免疫性溶血性贫血发作前已因输血而产生同种免疫；五名患者中有四名检测到两种或更多种同种特异性。自身抗体的存在显著影响了相容性检测；三名患者出现输血后血红蛋白尿，三次采用51Cr标记的供体红细胞进行体内交叉配血。所有患者对皮质类固醇均有反应；一名患者还接受了巯嘌呤治疗。所有患者出院后直接抗球蛋白试验结果均恢复为阴性。
