无基础疾病的CMV感染相关噬血细胞淋巴组织细胞增生症5例临床分析

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

回顾性分析2011年1月至2020年12月于南京医科大学第一附属医院确诊并治疗的5例巨细胞病毒(CMV)感染相关噬血细胞淋巴组织细胞增生症(HLH)患者的临床资料。5例患者中,女4例,男1例,中位年龄31岁(范围21~65岁)。患者发病前均无基础疾病且免疫功能健全。患者起病时均有发热,4例伴脾大,2例淋巴结肿大,1例肝大,3例伴皮疹。3例患者影像学证实肺部感染,其中2例患者出现呼吸衰竭。2例伴有黄疸。中枢神经系统症状合并消化道出血1例。所有患者均给予糖皮质激素以控制炎症因子风暴并使用抗病毒治疗。1例患者在抗病毒治疗无明显改善并出现中枢神经系统症状后,加用COP方案(环磷酰胺、长春新碱、泼尼松)化疗。经治疗,4例患者HLH获得完全缓解,1例妊娠合并HLH患者在自然分娩后最终因疾病进展死亡。无基础疾病的免疫健全患者CMV感染诱发HLH极为罕见。患者总体预后较好,抗病毒治疗为关键治疗手段。

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.