Objective To investigate the etiology, clinical characteristics, treatment and prognosis of hemophagocytic lymphohistiocytosis (HLH) in children. Methods The etiology, clinical characteristics, laboratory results, therapeutic effect and prognosis of HLH in 20 children were analyzed retrospectively. Results All the patients undergone a long-term hyperpyrexia at the first onset of HLH as well as the hepatosplenomegaly, half of them were complicated with lymphadenectasis. Peripheral cytopenia in two or three lineages and liver dysfunction were found in all patients. Besides, 17 cases (85% ) had coagulation abnormalities; 15 cases (75%) had hypertriglyceridemia; 13 cases (65%) had elevated serum ferritin ( SF) levels and 17 cases ( 85% ) had histiocytosis in bone marrow. As for the etiology of HLH , 12 patients (60%) were associated significantly with infection, and the others (40%) were caused by Epstein-Barr virus. During the treatment, 14 cases (70%) received irregular chemotherapy; 8 cases (40%) were complicated with hepatic injury and temperature fluctuation; 5 cases (25% ) died. Conclusion Childhood HLH is characterized with severe onset, rapid progression and high mortality. The patients often present with abnormalities in peripheral blood, bone marrow, liver function and the levels of triglyceride and SF. Early diagnostic treatment should be performed in the patients highly suspected with HLH follow HLH-2004.%目的 了解噬血细胞淋巴组织细胞增生症(HLH)的病因,临床特点,治疗及预后.方法 回顾性分析20例HLH患儿的发病相关因素,临床特点,实验室检查指标的变化,治疗效果以及转归.结果 所有病例均以持续高热起病,均有肝睥肿大,50%患儿有淋巴结肿大;所有患儿二系以上外周血细胞减少,肝功能畀常,凝血功能异常有17例(85%),高甘油三脂血症15例(75%),血清铁蛋白增高13例(65%).17例(85%)骨髓涂片可见组织细胞增生.12例(60%)和感染因素相关,其中8例(40%)明确与EB病毒感染有关.有6例(3%)按HLH-2004方案正规化疗,达到临床治愈.14例(70%)不规范化疗患儿,8例(40%)仍有肝功能异常和体温波动,5例(25%)死亡.结论 HLH起病急重,进展快,病死率高.有明显血象、骨髓细胞学、肝功能改变,高甘油三酯血症以及血清铁蛋白改变.对高度怀疑为HLH的患儿,应及早行诊断性治疗,规范按照HLH-2004方案化疗,改善预后.
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