Chuang M W, Lau Y M, Tung J N, Tung J C, Chen P M
Section of Hematology-Oncology, Sha-Lu Tung General Hospital.
Zhonghua Yi Xue Za Zhi (Taipei). 1991 Mar;47(3):208-12.
Erythropoietic hypoplasia occurring in the absence of abnormalities in the leukopoietic and thrombocytopoietic series is often defined as "pure red cell aplasia" (PRCA). This condition may appear as an acquired defect of either acute or chronic type, and a congenital form as well. The chronic form of acquired PRCA occurred mostly in adults. It has been reported that a demonstrable thymoma occurred in more than 50% of patients with PRCA. Recent studies suggested that it may contribute to several immune mechanisms. Here we report a case of thymoma with PRCA whose clinical presentations include severe anemia, shock with severe metabolic acidosis, high levels of several organ enzymes (SGOT, SGPT, LDH, CPK, Amylase) and acute renal shutdown with similar manifestations to septic shock. Our explanation to his condition is multi-organ tissue hypoxia caused by severe anemia. Hemophagocytic syndrome was found by the repeated bone marrow smear before death. The clinical course of this patient was so impressive as to be presented here for discussion.
在白细胞生成和血小板生成系列无异常的情况下发生的红细胞生成发育不全通常被定义为“纯红细胞再生障碍性贫血”（PRCA）。这种情况可能表现为急性或慢性类型的后天性缺陷，也有先天性形式。后天性PRCA的慢性形式多见于成年人。据报道，超过50%的PRCA患者存在可证实的胸腺瘤。最近的研究表明，它可能与多种免疫机制有关。我们在此报告一例伴有PRCA的胸腺瘤病例，其临床表现包括严重贫血、伴有严重代谢性酸中毒的休克、多种器官酶（谷草转氨酶、谷丙转氨酶、乳酸脱氢酶、肌酸磷酸激酶、淀粉酶）水平升高以及急性肾衰竭，其表现与感染性休克相似。我们对他病情的解释是严重贫血导致的多器官组织缺氧。在患者死亡前通过反复骨髓涂片发现了噬血细胞综合征。该患者的临床病程令人印象深刻，现予呈现以供讨论。
