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

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 cell could be even higher than normal, and with a significantly increased CRP. Autoimmune associated HLH often accompany with rash, usually without lowering of blood cells at the first visit, unconspicuous focus of infection, and antinuclear antibody is often positive. Therefore in clinical practice, we should identify HLH at an early stage, focusing on the primary disease, and thus effectively reducing mortality.