继发性噬血细胞性淋巴组织细胞增生症65例临床特点分析  

Analysis of clinical characteristics of 65 cases with secondary hemophagocytic lymphohistiocytosis

作　　者：罗丹[1] 黄丽芳[1] 曾雯[1] 苗宁宁[1] 刘文励[1] 黄伟[1] 周剑峰[1] 孙汉英[1] 

机构地区：[1]华中科技大学同济医学院附属同济医院,武汉430030

出　　处：《内科急危重症杂志》2015年第2期96-100,共5页Journal of Critical Care In Internal Medicine

摘　　要：目的:分析不同疾病所致继发性噬血细胞性淋巴组织细胞增生症(HLH)患者的临床特征,进一步加深对本病的认识。方法:回顾性分析65例继发性HLH患者的临床资料,将患者分为NK细胞淋巴瘤/白血病组、恶性肿瘤组、EB病毒相关组、风湿免疫相关组、重症感染组,比较5组患者的临床特点。结果:65例患者各种临床表现所占比例如下,100%发热,92.3%脾大,53.9%肝大,50.8%淋巴结肿大,30.8%存在水肿,20%出现皮疹。84.6%血细胞两系或三系减少,96.5%血清铁蛋白高,86.0%高甘油三酯血症,52.3%低纤维蛋白原血症,68.8%存在噬血现象,81.8%存在低或无NK细胞活性,93.9%肝酶异常,55.4%胆红素升高,96.9%低蛋白血症,72.3%低钠血症,96%高密度脂蛋白降低。5组患者在发热、脾大、淋巴结肿大、水肿、血细胞减少与否、血甘油三酯、铁蛋白水平、肝功能异常等方面无统计学差异(P>0.05);而在肝大、皮疹、初诊时血常规、纤维蛋白原、噬血现象等方面有统计学差异(P<0.05)。结论:继发性HLH有共同临床表现,如发热、脾大、血细胞减少、肝酶异常、低蛋白血症、低钠血症等,但不同疾病所致继发性HLH有一定的特点,EB病毒相关HLH表现为发病年龄小、肝大、血细胞明显减少、CRP高、噬血现象少见,经积极治疗后预后较好。重症感染相关HLH常表现为初诊时血细胞减少不明显,甚至高于正常,且CRP明显升高。风湿免疫相关HLH常有皮疹、初诊时血细胞常不减少、无明显感染灶、抗核抗体阳性。故在临床工作中,应针对不同病因早期识别HLH,从而有效降低病死率。Objective： To analyze the clinical features of hemophagocytic lymphohistiocytosis （ HLH ） secondary to differ- ent diseases, and further increase the understanding of this disease. Methods： Clinical data of 65 cases with secondary HLH were analyzed and compared retrospectively. Patients were divided into 5 groups ： NK cell lymphoma / leukemia group, malig- nant tumor group, EB virus related group, autoimmune associated group and severe infection group. Results： Clinical features of 65 patients were as follows ： 100% of the patients had fever, 92.3% had splenomegaly, 53.9% had hepatomegaly, and 50.8% of them had lymph node enlargement, 30.8% had edema, 20% had rash, 84.6% had two or three-lines cytopnea, 96.5% had high level of serum ferritin, 86.0% had hypertriglyeeridemia, 52.3% had hypofibrinogenemia, 68.8% had he- mophagoeytosis, low or no activity of NK cell were present in 81.8% of the patients, 93.9% showed abnormal liver transami- nase level, 55.4% with elevated bilirubin, 96.9% with hypoalbuminemia, 72.3% had hyponatremia, 96% had decreasing level of high-density lipoprotein. There were no significant differences in fever, splenomegaly, lymphadenopathy, edema, the level of blood cells, hypertriglyceridemia, high level of serum ferritin, abnormal liver function among five groups （ P 〉 0.05 ）. But in respect of hepatomegaly, rash, blood cell level at first visit, fibrinogen level, hemophagocytic phenomenon, the differ- ences were significant （P 〈 0.05）. Conclusions： The secondary HLH have some common clinical manifestations, such as fe- ver, splenomegaly, eytopenia, abnormal liver transaminase level, hypoalbuminemia, hyponatremia, etc. But different primary diseases have certain characteristics, such as EB virus related HLH shows young age onset, hepatomegaly, significant cytop- nea, high level of CRP, but rare hemophagocytosis. This type of HLH has a good prognosis after active treatment. In severe in- fection related HLH, the eytopenia was not obvious at first visit, the blood

关 键 词：噬血细胞性淋巴组织细胞增生症 继发性 临床特点 诊断 

分 类 号：R557.4[医药卫生—血液循环系统疾病]