Keefer M J, Solanki D L
Department of Medicine, University of Oklahoma Health Sciences Center, Oklahoma City.
Am J Hematol. 1988 Feb;27(2):132-5. doi: 10.1002/ajh.2830270212.
A 50-year-old woman with typical acquired primary pure red cell aplasia (PRCA) was successfully treated with prednisone. A later relapse was preceded by a period of ineffective erythropoiesis characterized by a reticulocyte response inappropriately low for the degree of anemia, serum iron of 189 micrograms/dl, total iron-binding capacity (TIBC) of 213 micrograms/dl, and erythroid hyperplasia. In addition there was marked dyserythropoiesis and erythroblast-phagocytosis. One month later, bone marrow examination showed classic PRCA. This rarely reported evolutionary stage of PRCA has several implications: 1) it suggests antibody induced erythroblast cytotoxicity as one mechanism of PRCA; 2) at a particular time in the development of PRCA there is potential for misdiagnosis as primary refractory anemia (PRA); and 3) some cases of PRA with similar morphologic and laboratory findings may be pathogenetically related to PRCA and may benefit from evaluation for immune-mediated suppression of erythropoiesis.
一名50岁患有典型获得性原发性纯红细胞再生障碍性贫血（PRCA）的女性患者，经泼尼松治疗成功。后来复发前有一段无效红细胞生成期，其特征为网织红细胞反应与贫血程度不相称地低、血清铁为189微克/分升、总铁结合力（TIBC）为213微克/分升以及红系增生。此外，有明显的红细胞生成异常和幼红细胞吞噬现象。1个月后，骨髓检查显示为典型的PRCA。这种PRCA罕见报道的演变阶段有几个意义：1）提示抗体诱导的幼红细胞细胞毒性是PRCA的一种机制；2）在PRCA发展的特定时期，有可能误诊为原发性难治性贫血（PRA）；3）一些具有相似形态学和实验室检查结果的PRA病例可能在发病机制上与PRCA相关，可能受益于免疫介导的红细胞生成抑制评估。
