噬血细胞综合征19例临床分析

Clinical characterizations of 19 patients with hemophagocytic syndrome

目的总结噬血细胞综合征(HPS)的临床特点。方法回顾性分析19例HPS合并肝损伤患者的病因、临床特点及转归。结果本组患者主要表现为持续高热(100.0%)、黄疸(73.7%)、胸腹腔积液(73.7%)、脾肿大(68.4%)、肝肿大(63.2%)、淋巴结肿大(57.9%),伴中枢神经系统症状(31.6%)及消化道出血(21.1%)。全部患者丙氨酸氨基转移酶(ALT)、天冬氨酸氨基转移酶(AST)明显升高[ALT202.6(9.9～1072.3)U/L,AST316.5(11.0～3578.1)U/L],以AST升高更为显著,伴乳酸脱氢酶升高;89.5%的患者总胆红素(TBil)明显升高。骨髓细胞学检查均发现有噬血现象。全部患者血清铁蛋白明显升高。89.5%的患者出现两系以上血细胞减少。HPS的原发病以感染性疾病为主(57.9%),原因不明者6例(31.6%)。病死率为57.9%。早期应用大剂量糖皮质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,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 syndrome （HI）S）. Methods A retro- spective study was carried out to analyze the underlying diseases, clinical characteristics and outcomes in 19 patients with HPS. Results HPS was clinically characterized by prolonged high-grade fever （100.0%）, jaundice （73.7%）, hydrops of multiple serous cavity （73.7%）, splenomegaly （68.4%）, hepatomegaly （63.2%）, lymphadenopathy （57.9%）, and other fea- tures including central nervous system abnormality （31.6%） and alimentary tract hemorrage （21.1%）. Laboratory data indi- cated that liver dysfunction was the most prominent feature （100.0%）, mainly manifested with dramatically increased level of TBil （89.5%）, AST, ALT, GGT and ALP. The other features were hemophagocytosis in bone marrow （100.0%）, high level of ferritin （100.0%）, peripheral eytopenias in two or more lineages （89.5%）. The underlying disease of infection was most common （57.9%）, unknown reason cases were 31.6%, systemic lupus erythematosus and lymphoma were also the causes of HPS （5.3% respectively）. The mortality was 57.9%. High doses of methylprednisolone and gamma globulin at the early phase of HPS could improve the outcome. Conclusion There are various underlying diseases and clinical manifestations of HPS. Prolonged high-grade fever and liver dysfunction are the prominent features of HPS. HPS is a severe disorder with poor prognos^s