28例噬血细胞性淋巴组织细胞增多症临床分析  

Clinical Analysis on 28 Patients with Hemophagocytic Lymphohistocytosis Syndrome

作　　者：舒汨汨[1] 朱华锋[1] 张涛[1] 高广勋[1] 陈协群[1] 

机构地区：[1]第四军医大学西京医院血液内科,陕西西安710032

出　　处：《中国实验血液学杂志》2010年第2期463-465,共3页Journal of Experimental Hematology

摘　　要：为了深入认识噬血细胞性淋巴细胞组织增多症(hemophagocytic lymphohistocytosis syndrome,HLH)的临床和实验室特征及疾病的诱因,对28例HLH患者进行了回顾性分析。结果表明,所有患者均有持续1周以上的发热、全血细胞减少、铁蛋白升高、低纤维蛋白原血症及肝功能障碍表现,部分患者尚有以下特点:9例HLH为恶性淋巴瘤的前期表现,1例以类似于暴发性肝功能衰竭的临床表现起病,1例以明显的精神异常为主要表现而在脑脊液中查见有明确的噬血细胞,1例因淋巴结活检发现噬血现象而确诊。另外,28例HLH的发生源自不同的诱因,其中EB病毒感染11例、杜氏利什曼原虫感染2例、淋巴瘤11例(以T细胞性淋巴瘤为主)、自身免疫病3例。结论:HLH临床表现与诱因具有高度异质性,来自牧区的患者需除外寄生虫感染(如利什曼原虫)的可能。In order to profoundly understand the clinical and laboratorial characteristics and inducing factors of hemophagocytic lymphohistocytosis syndrome （ HLH）, 28 HLH patients received from 2004 to 2009 years in our hispital were analyzed retrospectively. The results indicated that all of the patients had a history with prolonged fever （more than 1 week）, pancytopenia, hepatosplenomegaly, elevated ferritin level, hypofibrinogen, and hemophagocytosis in bone marrow. HLH was the first characteristic sign of malignant lymphoma in 9 patients; 1 patient had a clinical manifestation similar to fuiminant hepatic failure; severe psycho-abnorrnity occurred in 1 HLH patient and pronounced hemophagocytosis were detected in his cerebrospinal fluid; 1 patient was eventually diagnosed as having HLH by the findings in a lymph node biopsy showing obvious hemophagocytosis. Additionally, the analysis of underlying factors in 28 patients with HLH indicated 11 patients with EB virus-associated HLH, 11 with lymphoma-associated HLH, 2 with Leishmania-associated HLH, and 3 with autoimmune disease-associated HLH. It is concluded that HLH disease is characterised with high heterogenicity in both clinical features and inducing factors; in addition, the patients from a pasturing area should be paid attention to parasite infection such as leishinania.

关 键 词：噬血细胞淋巴组织增多症 EB病毒 杜氏利什曼原虫 淋巴瘤 

分 类 号：R557.4[医药卫生—血液循环系统疾病] R551.2[医药卫生—内科学]