儿童嗜血细胞综合征回顾性分析

A retrospective study of children with hemophagocytic syndrome

目的探讨儿童嗜血细胞综合征(HPS)的临床症状、实验室检测结果、治疗和预后,提高临床对该类疾病的认识。方法对本院2016年1月至2020年3月收治于儿童重症监护室(PICU)的17例HPS患儿的临床资料进行回顾性分析。结果17例HPS患儿最常见的临床症状为发热(100%)、肝肿大(52.94%)和皮疹(52.94%),其次为浅表淋巴结肿大(23.53%)、三凹征(17.65%)、脾肿大(11.76%)和黄疸(11.76%)。实验室检查中,血细胞两系或三系减少14例(82.35%)、铁蛋白升高17例(100%)、D-二聚体升高16例(94.12%)、血红蛋白降低16例(94.12%)、部分活化凝血活酶时间延长15例(88.24%)。均可见骨髓嗜血现象。检出自身免疫性疾病相关抗体5例,EB病毒阳性7例。经治疗,好转12例,死亡5例。结论HPS病情凶险进展迅速,但临床症状不特异且病因多样,临床医生应开拓诊断思维,动态观察各项检测指标,以达到早期诊断的目的。

Objective To investigate the clinical features,laboratory results,treatment and prognosis of children with hemophagocytic syndrome(HPS)for improving the clinical understanding of the disease Methods The clinical data of 17 children with HPS admitted to the pediatric intensive care unit(PICU)in Guangdong Women and Children Hospital from January 2016 to March 2020 were retrospectively analyzed.Results The most common clinical symptoms of the 17 children with HPS were fever(100%),hepatomegaly(52.94%)and rash(52.94%),followed by superficial lymphadenopathy(23.53%),tricavitary sign(17.65%),splenomegaly(11.76%)and jaundice(11.76%).In laboratory examination,14 cases(82.35%)of hemocyte secondary or tertiary lines decreased,17 cases(100%)of ferritin increased,16 cases(94.12%)of D-dimer increased,16 cases(94.12%)of hemoglobin decreased,15 cases(88.24%)of partially activated thromboplastin time prolonged.Hemophilic phenomenon of bone marrow was observed in all patients.There were 5 cases of autoimmune diseases related antibodies and 7 cases of EB virus positive.After treatment,12 patients recovered and 5 cases died.Conclusion Considering the complex clinical presentation and various etiology of children with HPS,dynamic observation of laboratory results and expansion of diagnostic strategy were needed to achieve the purpose of early diagnosis。