Ballas S K, Lewis C N, Noone A M, Krasnow S H, Kamarulzaman E, Burka E R
Am J Hematol. 1982 Aug;13(1):37-51. doi: 10.1002/ajh.2830130106.
Twenty-seven patients were seen and followed at our Sickle Cell Center over a period of seven years. Their clinical, hematological, and biochemical features were determined and compared to those of patients with sickle cell anemia who were concurrently investigated. The data indicate that the mild anemia of hemoglobin (Hb) SC disease is slightly microcytic and hyperchromatic. Parameters of hemolysis and the complications of chronic hemolytic anemia (cholelithiasis, leg ulcers, hepatomegaly, and cardiomegaly) are milder in Hb SC disease than in sickle cell anemia. Asplenia and its sequelae (increased platelet count and reduced serum IgM levels) are less frequent in Hb SC disease. Cerebrovascular accidents and the decreased leukocyte alkaline phosphatase scores are similar in both diseases. Thromboembolic complications, retinopathy, and renal papillary necrosis are more frequent in Hb SC disease.
在七年的时间里，我们的镰状细胞中心诊治并随访了27例患者。确定了他们的临床、血液学和生化特征，并与同期接受调查的镰状细胞贫血患者的特征进行了比较。数据表明，血红蛋白（Hb）SC病的轻度贫血呈轻度小红细胞性和高色素性。Hb SC病中溶血参数和慢性溶血性贫血并发症（胆石症、腿部溃疡、肝肿大和心脏肥大）比镰状细胞贫血中更轻。Hb SC病中脾切除及其后遗症（血小板计数增加和血清IgM水平降低）的发生率较低。两种疾病中脑血管意外和白细胞碱性磷酸酶评分降低的情况相似。Hb SC病中血栓栓塞并发症、视网膜病变和肾乳头坏死更为常见。
