成人嗜血细胞综合征的临床观察

Hemophagocytic Syndrome in Adults: A Report of 12 Cases and Literatures Review

目的探讨成人嗜血细胞综合征的病因、发病机制、临床表现、诊断、治疗及预后。方法 2011年2月-2013年10月,对12例嗜血细胞综合征(HPS)患者的病因、临床表现、治疗及预后结合文献进行分析。结果临床表现:发热12例(100.0%),肺部感染10例(83.3%),淋巴结肿大6例(50.0%),出血表现3例(25.0%),脾肿大2例(16.7%),肝肿大1例(8.3%)。实验室检查:骨髓嗜血现象12例(100.0%),铁蛋白升高11例(91.7%),血小板降低12例(100.0%),白细胞降低10例(83.3%),纤维蛋白原降低8例(66.7%),高甘油三酯血症7例(58.3%)。10例感染相关性HPS,2例弥漫大B细胞淋巴瘤相关性HPS。2例治愈,9例死亡,1例自动出院。结论 HPS临床罕见,感染相关性HPS最常见,临床表现多样,可累及多个系统,病情发展迅速,治疗难度大,病死率高。

Objective To study the etiology, pathogenesis, clinical manifestations, diagnosis, treatment and prognosis of hemophagocytic syndrome （HPS）. Methods Between February 2011 and October 2013, the causes, clinical manifestations, treatment and prognosis of 12 cases of HPS were retrospectively analyzed, and related literatures were reviewed. Results There were 4 males and 8 females aged from 23 to 79 years old. Clinical features included fever （100.0%）, lung infection （83.3%）, enlarged lymph nodes （50.0%）, bleeding （25.0%）, splenomegaly （16.7%）, and hepatomegaly （8.3%）. Laboratory test results showed hemophagocytosis in bone marrow （100.0%）, elevated ferritin （91.7%）, thrombocytopenia （100.0%）, decreased count in white blood cells （83.3%）, decreased fibrinogen （66.7%）, and hypertriglyceridemia （58.3%）. Ten cases were related with infection, and 2 were related with diffused large B cell lymphoma. Nine patients died, 2 were cured, and one discharged himself from the hospital. Conclusions HPS is an uncommon disease, in which infection-associated HPS is the most common. The clinical presentation is complex, usually associated with multi-organ dysfunction, aggressive course, and high mortality rate.