摘要
目的提高对噬血细胞综合征(HPS)的临床表现和治疗的认识。方法对我中心收治的10例HPS病儿的病因、临床表现、诊断、治疗及预后进行分析。结果 10例HPS病儿有5例与感染有关,1例为急性淋巴细胞白血病所致,4例病因未明。本组病例主要表现为持续发热,肝大及脾大,实验室检查以血常规二系或三系减少、肝功能损害为主,其次为骨髓有噬血细胞现象、血清铁蛋白升高、高三酰甘油血症、低纤维蛋白原血症等。积极治疗原发病,同时给予免疫抑制剂及对症支持治疗,好转5例,死亡2例,自动出院3例。结论 HPS的临床表现复杂多样,病情凶险,病死率高,多次骨髓检查有助于及时诊断,早期诊断、早期治疗是提高病儿生存率的关键。
Objective To better know the clinical manifestations and treatment of hemophagocytic syndrome（HPS）.Methods Ten children with HPS admitted to our hospital,between May 2008 and March 2011,were studied.Their etiological factors,clinical manifestations,diagnosis,therapy and prognosis were analyzed.Results Of the 10 cases,five were related with infection,one was caused by acute lymphocytic leukemia,and four of unknown origin.The main manifestations of HPS in this group were continuing fever,and hepatosplenomegaly.Laboratory data indicated that liver dysfunction and decrease of 3 or 2 blood cell lines was the most prominent feature,and then were hemophagocytosis in bone marrow,hyperferritinemia,hypertriglyceridemia,and hypofibrinogenmial.After treatment with immunodepressant and combined therapy,five cases improved,two died and three voluntarily discharged from hospital.Conclusion Clinical manifestations of HPS are various and complicated,with dange-rous condition and high mortality.Repeated bone marrow examinations are conducive to a diagnosis in time.An early diagnosis and treatment is the key to raise the survival rate of sick children with this disease.
出处
《齐鲁医学杂志》
2012年第1期73-74,共2页
Medical Journal of Qilu
