Saha M, Ray S, Kundu S, Chakrabarti P
Department of Medicine, Medical College and Hospital, Kolkata, West Bengal, India.
J Postgrad Med. 2013 Jan-Mar;59(1):51-3. doi: 10.4103/0022-3859.109495.
A 26-year-old previously healthy female presented with a 6-month history of anemia. The laboratory findings revealed hemolytic anemia and direct antiglobulin test was positive. With a diagnosis of autoimmune hemolytic anemia (AIHA), prednisolone was started but was ineffective after 1 month of therapy. A bone marrow trephine biopsy revealed pure red cell aplasia (PRCA) showing severe erythroid hypoplasia. The case was considered PRCA following AIHA. This combination without clear underlying disease is rare. Human parvovirus B19 infection was not detected in the marrow aspirate during reticulocytopenia. The patient received azathioprine, and PRCA improved but significant hemolysis was once again documented with a high reticulocyte count. The short time interval between AIHA and PRCA phase suggested an increased possibility of the evolution of a single disease.
一名26岁既往健康的女性出现了6个月的贫血病史。实验室检查结果显示为溶血性贫血,直接抗人球蛋白试验呈阳性。诊断为自身免疫性溶血性贫血(AIHA)后,开始使用泼尼松龙治疗,但治疗1个月后无效。骨髓环钻活检显示纯红细胞再生障碍(PRCA),表现为严重的红系造血低下。该病例被认为是AIHA后发生的PRCA。这种无明确潜在疾病的组合很罕见。在网织红细胞减少期间,骨髓穿刺物中未检测到人细小病毒B19感染。患者接受了硫唑嘌呤治疗,PRCA有所改善,但再次出现显著溶血,网织红细胞计数升高。AIHA和PRCA阶段之间的时间间隔较短,提示单一疾病演变的可能性增加。