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目的:探讨儿童噬血细胞综合征(white blood cell syndrome,HPS)的临床特点、诊断标准、治疗过程以及预后的相关危险因素。方法:回顾分析2009—2013年南京市儿童医院血液肿瘤科收治的72例儿童HPS的临床表现、辅助检查、治疗及转归,采用Logistic方法分析患儿的预后危险因素。结果:72例中42例(58.3%)是感染相关性HPS,其中EB病毒感染相关性HPS最多;其余的30例中4例(5.56%)是非感染相关HPS,26例(36.1%)病因不明。临床表现为发热(91.7%)、肝脏肿大(83.3%)、脾脏肿大(66.7%)。外周血血常规三系检查结果为两系减低(72.2%),三系低下(27.8%),血红蛋白浓度减低(91.7%),血小板数量减少(77.8%);血生化检查血清甘油三酯升高(38.9%);凝血常规检查功能障碍(41.7%);血清铁蛋白升高(55.6%);骨髓细胞形态学检查大部分找到了噬血细胞。72例中死亡10例(13.8%),好转或者基本痊愈24例(33.3%),出院后失访14例(19.4%)。年龄<3岁、乳酸脱氢酶(LDH)>2 500 U/L是不利的危险因素。结论:年龄小和LDH水平增高是儿童HPS预后不良的危险因素,应该及早诊断治疗,降低病死率。
Objective: To investigate the clinical features, diagnostic criteria, treatment course and prognostic risk factors of childhood hemophagocytic syndrome (HPS). Methods: The clinical manifestations, auxiliary examinations, treatment and prognosis of 72 children admitted to Department of Hematology and Oncology, Nanjing Children’s Hospital from 2009 to 2013 were retrospectively analyzed. Logistic method was used to analyze the prognostic risk factors in children. Results: Of the 72 cases, 42 (58.3%) were HPS infected, of which HPS was most associated with Epstein-Barr virus infection; 4 (5.56%) of the remaining 30 were non-HPS-infected and 26 (36.1%) had unknown etiology . The clinical manifestations were fever (91.7%), enlarged liver (83.3%) and enlarged spleen (66.7%). In the peripheral blood, the three-line blood test results showed that the blood was lower in two lines (72.2%), lower in three lines (27.8%), hemoglobin concentration was reduced (91.7%), and the number of platelets was decreased (38.9%); coagulation routine examination dysfunction (41.7%); elevated serum ferritin (55.6%); most of the bone marrow cell morphological examination found hemophagocytic cells. Of the 72 patients, 10 died (13.8%), 24 cases (33.3%) recovered or basically recovered, and 14 patients (19.4%) lost their follow-up after discharge. Age <3 years, lactate dehydrogenase (LDH)> 2 500 U / L is a negative risk factor. Conclusion: Small age and elevated LDH levels are risk factors for poor prognosis of children with HPS. Early diagnosis and treatment should be performed to reduce the mortality.