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5例无基础疾病的巨细胞病毒感染相关噬血细胞性淋巴组织细胞增生症临床分析

[Clinical analysis of cytomegalovirus infection-associated hemophagocytic lymphohistiocytosis in five patients without underlying diseases].

作者信息

Zhang X Y, Qiu T L, Huang H J, Zhu H Y, Li J Y, Shi W Y, Miao Y

机构信息

Department of Hematology, the First Affiliated Hospital of Nanjing Medical University (Jiangsu Province Hospital), Nanjing 210029, China.

Department of Oncology, Affiliated Hospital of Nantong University, Nantong 226001, China.

出版信息

Zhonghua Nei Ke Za Zhi. 2024 May 1;63(5):486-489. doi: 10.3760/cma.j.cn112138-20230920-00146.

Abstract

The clinical data of five patients [one male and four female; median age: 31 (21-65) years] with cytomegalovirus (CMV)-induced hemophagocytic lymphohistiocytosis (HLH) diagnosed and treated in the First Affiliated Hospital of Nanjing Medical University were retrospectively analyzed from January 2011 to December 2020. None of the patients had any underlying disease, and all were immunocompetent. The main clinical presentations were fever in all five patients, splenomegaly in four, enlarged lymph nodes in two, liver enlargement in one, and rash in three. Pulmonary infection was found in three patients, two of whom developed respiratory failure. Two patients had jaundice. Central nervous system symptoms and gastrointestinal bleeding were observed in one case. All patients received glucocorticoids and antiviral therapy. One patient was treated with the COP (cyclophosphamide+vincristine+prednisone) chemotherapy regimen after antiviral therapy failed and he developed central nervous system symptoms. After treatment, four patients achieved remission, but the fifth pregnant patient eventually died of disease progression after delivery. CMV-associated HLH in an immunocompetent individual without underlying diseases is extremely rare, and most patients have favorable prognosis. Antiviral therapy is the cornerstone of CMV-HLH treatment.

摘要

回顾性分析2011年1月至2020年12月在南京医科大学第一附属医院诊断和治疗的5例巨细胞病毒(CMV)诱导的噬血细胞性淋巴组织细胞增生症(HLH)患者的临床资料。5例患者均无基础疾病,免疫功能正常。主要临床表现为5例患者均有发热,4例脾肿大,2例淋巴结肿大,1例肝脏肿大,3例皮疹。3例患者发生肺部感染,其中2例发展为呼吸衰竭。2例患者出现黄疸。1例出现中枢神经系统症状和胃肠道出血。所有患者均接受糖皮质激素和抗病毒治疗。1例患者抗病毒治疗失败后接受COP(环磷酰胺+长春新碱+泼尼松)化疗方案治疗,出现中枢神经系统症状。治疗后,4例患者缓解,但第5例孕妇最终在分娩后因疾病进展死亡。无基础疾病的免疫功能正常个体中CMV相关HLH极为罕见,大多数患者预后良好。抗病毒治疗是CMV-HLH治疗的基石。

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