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目的探讨噬血性淋巴组织细胞增生症(HLH)的临床特点。方法回顾性分析2009年4月-2010年5月本院血液科收治的20例HLH患儿临床表现及血常规、肝功能、血液生化、病原学、免疫学检测、血清铁蛋白及骨髓涂片检查等实验室指标,参照HLH-2004治疗方案治疗。结果患儿主要表现为持续发热,肝、脾、淋巴结大,全血细胞减少,肝功能受损,凝血障碍,低纤维蛋白血症,高三酯甘油血症,自然杀伤细胞比例降低。12例中性粒细胞<1.0×109L-1,16例Hb<90 g.L-1,18例血小板<100×109L-1,血细胞二系降低者18例,三系同时降低12例。ALT升高20例,AST升高18例,LDH升高20例,胆碱酯酶升高13例。三酰甘油升高18例,血清铁蛋白>1500μg.L-18例,ESR增快9例;CRP增高11例,自然杀伤细胞比例降低12例,骨髓增生活跃17例,增生低下3例,12例骨髓涂片找到噬血细胞。20例发病与感染相关者中,与EB病毒感染相关性噬血细胞综合征3例,巨细胞病毒感染相关4例,铜绿假单胞菌感染1例。治愈3例,好转15例,疗效不佳自动出院2例。结论 HLH临床表现多样,及时诊治可改善预后。
Objective To investigate the clinical features of hemophagocytic lymphohistiocytosis (HLH). Methods The clinical manifestations, blood tests, liver function, blood biochemistry, etiology, immunological tests, serum ferritin and bone marrow smears of 20 children with HLH admitted to our hospital from April 2009 to May 2010 were retrospectively analyzed. Laboratory tests such as indicators, with reference to HLH-2004 treatment programs. Results The main manifestations of children with persistent fever, liver, spleen, lymph nodes, pancytopenia, impaired liver function, coagulation disorders, hypofibrinogenemia, hypertriglyceridemia, natural killer cells decreased. 12 cases of neutrophils <1.0 × 109L-1, 16 cases of Hb <90 g.L-1, 18 cases of platelets <100 × 109L-1, 18 cases of secondary reduction of blood cells, 12 cases of three-line reduction at the same time. ALT increased in 20 cases, AST increased in 18 cases, elevated LDH in 20 cases, elevated cholinesterase in 13 cases. Elevated triglyceride was found in 18 cases, serum ferritin> 1500 μg. L-18 cases, and ESR increased in 9 cases. CRP increased in 11 cases, natural killer cells decreased in 12 cases, bone marrow hyperplasia in 17 cases, hyperplasia in 3 cases, Case of bone marrow smear to find hemophagocytic cells. Among the 20 cases of pathogenesis and infection, 3 cases were associated with Epstein-Barr virus infection hemophagocytic syndrome, 4 cases were associated with cytomegalovirus infection and 1 case was infected with Pseudomonas aeruginosa. 3 cases were cured, 15 cases improved, 2 cases were discharged automatically with poor curative effect. Conclusion The clinical manifestations of HLH are diverse and timely diagnosis and treatment can improve the prognosis.