Xu Lu-Hong, Fang Jian-Pei, Weng Wen-Jun, Huang Ke, Guo Hai-Xia, Liu Yong, Zhang Jin-Hua
Department of Pediatrics, Sun Yat-sen Memorial Hospital, Sun Yat-sen University, Guangzhou, China.
Hematology. 2013 Jan;18(1):56-9. doi: 10.1179/1607845412Y.0000000044. Epub 2012 Nov 19.
Pure red cell aplasia (PRCA) associated with cytomegalovirus (CMV) and Epstein-Barr virus (EBV) infection is uncommon. Here, we describe the clinical features and management of seven cases of Chinese children with PRCA associated with viral infections. The patients presented with pallor on admission. Blood cell counts and marrow smears showed anemia, reticulocytopenia, and aplasia of erythroblasts. Serological investigation and DNA polymerase chain reactions for CMV were positive in four patients and those tests for EBV were positive in other three patients. All patients received blood transfusion, corticosteroids treatment, and ganciclovir injection. Two patients had a complete response and one had a partial response after the treatments. The other three patients had a complete response to second-line therapies, including high-dose methylprednisolone, cyclosporin A, and intravenous immunoglobulin. Only one patient had no response to the therapies. Our results indicated that it might be important to combine immunosuppressive drugs with an antiviral drug in the management of PRCA associated with CMV and EBV infection.
与巨细胞病毒(CMV)和爱泼斯坦-巴尔病毒(EBV)感染相关的纯红细胞再生障碍性贫血(PRCA)并不常见。在此,我们描述了7例与病毒感染相关的PRCA中国儿童患者的临床特征及治疗情况。患者入院时表现为面色苍白。血细胞计数和骨髓涂片显示贫血、网织红细胞减少以及成红细胞再生障碍。4例患者CMV血清学检查及DNA聚合酶链反应呈阳性,另外3例患者EBV相关检查呈阳性。所有患者均接受了输血、皮质类固醇治疗及更昔洛韦注射。治疗后2例患者完全缓解,1例部分缓解。另外3例患者对包括大剂量甲泼尼龙、环孢素A及静脉注射免疫球蛋白在内的二线治疗完全缓解。仅1例患者对治疗无反应。我们的结果表明,在治疗与CMV和EBV感染相关的PRCA时,将免疫抑制药物与抗病毒药物联合使用可能很重要。