Sekiguchi Yasunobu, Shimada Asami, Imai Hidenori, Wakabayashi Mutsumi, Sugimoto Keiji, Nakamura Noriko, Sawada Tomohiro, Komatsu Norio, Noguchi Masaaki
Department of Hematology, Juntendo University Urayasu Hospital Japan.
Shibanishi Clinic Japan.
Int J Clin Exp Pathol. 2014 Apr 15;7(5):2624-35. eCollection 2014.
The patient was a 47-year-old man diagnosed as having autoimmune hemolytic anemia (AIHA) in April 2011. He also had a congenital chromosomal abnormality, a balanced translocation. Treatment with prednisolone (PSL) 60 mg/day resulted in resolution of the AIHA, and the treatment was completed in November 2011. While the patient no longer had anemia, the direct and indirect Coombs tests remained positive. In May 2013, he developed recurrent AIHA associated with acute pure red cell aplasia (PRCA) and hemophagocytic syndrome (HPS) caused by human parvovirus B19 (HPV B19) infection. Tests for anti-erythropoietin and anti-erythropoietin receptor antibodies were positive. Steroid pulse therapy resulted in resolution of the AIHA, PRCA, as well as HPS. The serum test for anti-erythropoietin antibodies also became negative after the treatment. However, although the serum was positive for anti-HPV B19 IgG antibodies, the patient continued to have a low CD4 lymphocyte count (CD4, <300/μL) and persistent HPV B19 infection (HPV B19 DNA remained positive), suggesting the risk of recurrence and bone marrow failure.
该患者为一名47岁男性，于2011年4月被诊断为自身免疫性溶血性贫血（AIHA）。他还患有先天性染色体异常，即平衡易位。使用泼尼松龙（PSL）60mg/天进行治疗后，AIHA得到缓解，治疗于2011年11月完成。虽然患者不再贫血，但直接和间接抗人球蛋白试验仍为阳性。2013年5月，他出现了与人类细小病毒B19（HPV B19）感染引起的急性纯红细胞再生障碍性贫血（PRCA）和噬血细胞综合征（HPS）相关的复发性AIHA。抗促红细胞生成素和抗促红细胞生成素受体抗体检测呈阳性。类固醇冲击疗法使AIHA、PRCA以及HPS均得到缓解。治疗后抗促红细胞生成素抗体的血清检测也转为阴性。然而，尽管血清抗HPV B19 IgG抗体呈阳性，但患者的CD4淋巴细胞计数持续偏低（CD4，<300/μL），且HPV B19感染持续存在（HPV B19 DNA仍为阳性），提示有复发和骨髓衰竭的风险。
