15例嗜血细胞综合征合并肝损伤的临床分析

Clinical analysis of 15 patients with hemopagocytic syndrome

目的分析嗜血细胞综合征（HPS）合并肝损伤的临床特点。方法对我院2006年1月至2010年12月就诊的15例HPS合并肝损伤患者的病因、临床特点及转归进行分析。结果本组病例发病以持续高热（100．O％）、浆膜腔积液（80．0％）、黄疸（80．O％）、脾肿大（73．3％）、肝肿大（66．7％）、淋巴结肿大（60．O％）为主要表现，可有中枢神经系统症状（26．7％）及消化道出血（20．O％）。肝脏损害最为突出（100．O％），表现为总胆红素（TBil）、ALT、AST、γ-谷氨酰转移酶和碱性磷酸酶均明显升高，胆碱胆酶及白蛋白下降。15例患者均发现骨髓中有噬血现象，患者铁蛋白明显升高，两系以上血细胞减少（93．3％）也较常见，常呈小细胞低色素性贫血；另外，甘油三酯升高（70．0％），纤维蛋白原下降（64．3％）。3例患者血清可溶性IL-2受体（sCD25）水平升高、NK细胞活性降低。病因分析显示，感染相关性HPS占多数（53．3％），淋巴瘤及系统性红斑狼疮各1例，占6．7％。9例死亡，病死率为60．0％。早期应用大剂量激素或丙种球蛋白可改善预后。结论HPS可由多种病因所致，临床表现复杂多样，持续发热和肝功能损伤是最突出的临床表现，预后不良。

Objective To investigate the clinical features of hemophagocytic syndrorne（HP3） with liver injury. Methods To analyze the clinical characteristics and outcomes in 15 patients with HPS, who enrolled in our hospital faun January 2006 to Deccmber 2010. HPS was clinically cboracterized by prolonged high-grade fever （ 100.0% ）, bydrops of multiple serous cavity （80.0%）, jaundice （80.0%）, splenomegaly （73.3%）, hepatomegaly （66.7%）, and lymphadenopathy （60.0%）, and other features including central nervous system abnennality （26.7%） and alimen- tary tract hereon-age（20.0% ）. Laboratory data indicated that liver injury was the most prominent feature （ 100.0% ）, mainly manifested with dramatically increased level of total bilirubin, ASF, ALT, γ-GT and ALP, and with decreased level of CHE and ALB. All the patients were with hemophagocytosis in bone marrow and high level of ferritin, some were with peripheral cytopenias in two or more lineages （93.3%）, hypertriglyceridemia （70.0%）, and hypofibrinogonemia （64.3%）. High concentration of serum soluble CD25 and low NK cell activity could be found in 3 pntients who got the tests. The disease with infection was most common （53.3%）, Systemic lupus erythematosus and lymphoma were also the causes of liPS （6.7%）. Nine patientis were dead and the mortality was 60.0%. High dose of methylprednisolone and gamma globulin at the early phase of HPS could improve the outcome. Conclusions There are various underlying disease and clinical manifestations of HPS. Prolonged high-grade fever and liver dysfunction are the prominent features of I-IPS. HPS is a severe disorder with poor prognosis.