Choudhry V P, Saraya A K, Kasturi J, Rath P K
Acta Haematol. 1981;66(3):195-201. doi: 10.1159/000207119.
3 cases of congenital dyserythropoietic anaemia (type I, case 3; type III, cases 1 and 2) are reported with a follow-up of 8-10 years. Conventional therapy with vitamins, steroids and/or anabolic hormones was ineffective. Regular blood transfusions were required to maintain the haemoglobin levels around 8 g/dl. Splenectomy, however, resulted in a sustained rise in haemoglobin without blood transfusions. During the follow-up period varying from 40 to 77 months after splenectomy, diabetes mellitus was detected in all 3 patients. Liver biopsy showed haemosiderosis in 2 patients with cirrhosis in one of them. It is suggested that splenectomy could be an effective mode of therapy to reduce the need for blood transfusion in congenital dyserythropoietic anaemias.
报告了3例先天性红细胞生成异常性贫血（I型，病例3；III型，病例1和2），随访时间为8至10年。使用维生素、类固醇和/或合成代谢激素的传统疗法无效。需要定期输血以将血红蛋白水平维持在8 g/dl左右。然而，脾切除术后血红蛋白持续上升，无需输血。在脾切除术后40至77个月的随访期间，所有3例患者均检测出糖尿病。肝活检显示2例患者有含铁血黄素沉着症，其中1例有肝硬化。提示脾切除术可能是减少先天性红细胞生成异常性贫血输血需求的一种有效治疗方式。
