儿童噬血细胞综合征32例临床分析  

Hemophagocytic syndrome in 32 children

作　　者：李小琳[1] 刘玉玲[1] 付四毛[1] 

机构地区：[1]南方医科大学附属中山市博爱医院儿内科,528400

出　　处：《中国小儿急救医学》2012年第1期38-40,共3页Chinese Pediatric Emergency Medicine

摘　　要：目的探讨儿童噬血细胞综合征（hemophagocytic syndrome，HPS）的临床表现、病因、诊断及治疗。方法回顾性分析我科收治的32例HPS患儿的病因、临床症状、体征、实验室检查结果及治疗转归情况。结果（1）病因：全部病例中病毒感染相关性HPS占75．0％（24／32），其中以EB病毒感染最为常见，占68．8％（22／32）。（2）临床表现：主要为持续发热，肝、脾及淋巴结肿大。（3）实验室检查特点：外周血常规表现为三系或二系减少，血清铁蛋白明显增高，肝功能受损，三酰甘油升高和（或）纤维蛋白原降低，有凝血障碍。骨髓涂片可找到噬血细胞，可溶性白细胞介素-2受体增高，自然杀伤细胞活性降低。（4）治疗及转归：32例HPS患儿中，24例病毒感染相关性HPS患儿均予抗病毒药物、大剂量人血丙种球蛋白治疗。22例予糖皮质激素、环磷酰胺等治疗，好转11例，完全缓解5例，死亡4例，疗效不佳自动出院2例；另外10例采用HLH-2004方案治疗，好转4例，完全缓解6例。结论HPS多由感染尤其是EB病毒感染所诱发，血清铁蛋白、可溶性白细胞介素-2受体及自然杀伤细胞活性可早期反映疾病转归，化疗过程中应定期监测。HLH-2004方案是HPS有效的治疗方案。Objective To explore the clinical features, etiological factors, diagnosis and treatments in children with hemophagocytic syndromes （HPS）. Methods A retrospective study was carried out to analyze etiological factors, clinical symptoms and sighs,laboratory findings and outcomes of 32 children with HPS in our hospital. Results （ 1 ） Etiological factors： virus infection cases accounted for 75.0% （24/32）, in which Epstein-Barr virus infection cases were predominant,making up to 68.8% （22/32） ; （2） Main clinical features ：included persistent fever, hepatosplenomegaly, lymphadenopathy ; （ 3 ） Characteristic laboratory findings：included decrease of three lines or two lines of peripheral blood cells, hyperferritinemia, abnormal liver function and coagulation function, hypertriglycerdema and decreased fibrinogen,hemophagocytosis in bone marrow,high soluble interleukin-2 receptor level, decreased natural killer cell activity. （4） Treatments and outcomes ：twenty-four （24/32） virus infection cases received antivirus and high dose of immunoglobulin treatment. Twenty-two cases were treated with glucocorticoid and cyclophosphamide,resulting in 11 cases improve- ment,5 cases with complete remission,2 cases giving up and 4 death. The other 10 cases were treated under the guide of I-ILH-2004 protocol treatmemt with 4 cases improved and 6 cases with complete remission. Conduslon HPS is mainly triggered by infections, especially Epstein-Barr virus infection. Serum ferritin,interleukin-2 receptor level and natural killer cell activity are biomarkers of the disease, so that they should be monitored during the progress of chemotherapy. HLH-2004 protocol is a good guide for the treatment of this disease.

关 键 词：噬血细胞综合征 EB病毒 血清铁蛋白 白细胞介素-2受体 自然杀伤细胞 儿童 

分 类 号：R725.5[医药卫生—儿科]