HIV阴性儿童马尔尼菲青霉病并发噬血细胞综合征1例及文献复习

Non-HIV children suffered from disseminated penicilliosis marneffei accompanying with hemophagocylic syndrome:1 case report and literature review

目的探讨HIV阴性儿童播散性马尔尼菲青霉菌病(DPSM)并发噬血细胞综合征(HPS)的临床特征。方法回顾性分析1例HIV阴性儿童DPSM并发HPS的病例资料,并复习相关文献,总结其临床特征及诊治经验。结果患儿男,2岁7个月,临床主要表现为发热、咳嗽、面色苍白、肝脾肿大,辅助检查提示全血细胞减少,低纤维蛋白原血症,转氨酶升高,铁蛋白升高,EBV-DNA载量升高,HIV抗体阴性,血液及骨髓培养检出马尔尼菲青霉菌,骨髓涂片可见噬血现象及吞噬孢子,肺部CT提示多发结节、空洞,符合噬血细胞综合征及播散性马尔尼菲青霉病诊断标准;治疗上予HLH-2004方案,同时给予伊曲康唑联合两性霉素B脂质体抗真菌及支持等综合处理,病情一度好转,但反复,最终因呼吸衰竭死亡。结论 HIV阴性儿童DPSM并发HPS,临床上少见且表现缺乏特异性,病情进展快,预后差;早期诊断,早期治疗,可能改善预后。

Objective This study was to discuss the clinical features of non-HIV children suffered from disseminated penicilliosis marneffei （DPSM） accompanying with hemophagocytic syndrome （HPS）.Methods We retrospectively analyzed the clinical manifestations of a non-HIV child with DPSM.Then we reviewed the related literature and summarized the clinical manifestations and the experience.Results The patient,a 2-year,7-month-old boy,suffered from fever,cough,pallor and hepatosplenomegaly.He had cytopenias,hypofibrinogenemia,elevated aminotransferase and ferritin,overload of EBV-DNA and negative HIV antibody.When we cultured his various body fluids for fungus pathogens,we found that penicillium marneffei had been growing in peripheral blood and marrow fluids.Marrow smears showed hemophagocytosis and spores.CT scans of the lungs showed multiple nodules and cavities.The above clinical manifestations accorded with the diagnostic criteria for HPS and DPSM.He was therefore provided with therapy according to HLH-2004,itraconazole combined with liosomal amphotericin B antifungual and supportive treatment.His condition was improved once.But illness was recurrent,and he eventually died of respiratory failure.Conclusions Non-HIV children suffered frown DPSM accompanying with HPS were rare and with bad prognosis,its clinical manifestations were nonspecific.Early identification and timely treatment was significant for improving the prognosis.