Hanna M D, Mattheis P J
McKennan Hospital, Sioux Falls, SD.
S D J Med. 1990 Dec;43(12):11-4.
The case of erythroblastopenia in a nine-year old boy who was healthy prior to the rapid onset of anemia is presented. There was no previous personal or family history of anemia or congenital anomaly. The initial Hgb was 4.0 gram/dl requiring the need for PRBC transfusions; the MCV was elevated throughout the course of disease. Continued erythroblastopenia after 8 weeks of observation prompted treatment with prednisone to which the anemia rapidly responded; and hematologic values have remained normal more than 1 1/2 years after discontinuation of steroid therapy. The case is discussed in the context of a diagnostic differentiation between transient erythroblastopenia of childhood (TEC) and Diamond-Blackfan Anemia (DBA); since it does not clearly fit either category it could either be an unusual TEC presentation with extended macrocytic anemia dependent on steroid therapy for remission, or a late onset DBA with potential for eventual relapse.
本文报告了一名9岁男孩的红细胞生成减少症病例，该男孩在迅速发生贫血之前身体健康。既往无个人或家族贫血史或先天性异常史。初始血红蛋白（Hgb）为4.0克/分升，需要输注浓缩红细胞；在疾病过程中平均红细胞体积（MCV）一直升高。观察8周后持续存在红细胞生成减少，促使使用泼尼松治疗，贫血对此迅速产生反应；在停用类固醇治疗后超过1年半的时间里，血液学指标一直保持正常。本文结合儿童暂时性红细胞生成减少症（TEC）和先天性纯红细胞再生障碍性贫血（DBA）的诊断鉴别来讨论该病例；由于该病例不符合这两种类型中的任何一种，它可能要么是一种不寻常的TEC表现，伴有依赖类固醇治疗缓解的持续性大细胞贫血，要么是一种迟发性DBA，有最终复发的可能。
