反应性嗜血细胞综合征临床特征分析

目的总结反应性嗜血细胞综合征(RHS)的临床特征、实验室资料、治疗及预后。方法分析22例诊断为RHS病人的临床特点、实验室检查和治疗方案。同时以骨髓细胞学证实发生嗜血细胞与否分组, 比较2组之间的异同情况。结果主要临床特征为高热发生率95.5%, 肝脾、淋巴结肿大发生率81.8%，血液系统受累、贫血发生率72.7%，血小板减少50.0%，RHS发生时WBC(19.5±8.1)×109/L，血红蛋白(95.2±14.4)g/L，白蛋白(26.1±4.4)g/L，三酰甘油(3.2±2.1)mmol/L，纤维蛋白原(1.5±0.7)g/L。经骨髓细胞学明确见到嗜血现象11例，嗜血组和非嗜血组病人实验室检查各项指标阳性率差异均无统计学意义(P>0.05)。50.0%(11/22)病人经治疗后缓解出院(其中1例失访)，病死10例，其中成人Still病5例，淋巴瘤1例，皮肌炎1例，抗中性粒细胞胞质抗体相关性血管炎1例，系统性红斑狼疮1例，肠系膜脂膜炎1例。呼吸衰竭是主要死因。结论风湿性疾病导致的RHS以成人still病多见，其他RHS最常见的为淋巴瘤，该病预后差, 死亡发生率较高，呼吸衰竭为主要死因。抓住实验室相关指标的动态变化有助于RHS的早期识别。激素联合环磷酰胺可控制疾病的进程。

Analysis of the clinical characteristics of reactive hemophagocytic syndrome

ObjectiveTo analyze the clinical presentation, laboratory data, treatment and prognosis of reactive hyemophagocytic syndrome(RHS).MethodsThe clinical presentation, laboratory test, treatment protocols in 22 cases with RHS were retrospectively analyzed.The patients were divided into two groups according to the bone marrow hemocytology, and the difference between two groups was compared.ResultsAmong 22 patients, the incidence rates of hyperpyrexia, lymphadenectasis, blood system involovement rate, anemia and thrombocytopenia were 95.5%, 81.8%, 72.7% and 50.0%, respectively.The levels of WBC, HB, ALB, TG and fibrinogen(Fbg) in RHS patients were(19.5±8.1)×109/L, (95.2±14.4)g/L, (26.1±4.4)g/L, (3.2±2.1)mmol/L and(1.5 ±0.7)g/L, respectively.The bone marrow cytology Results showed the hemophagocytic phenomenon in 11 cases were indentified, and the difference of the positive indicators of examination between two groups were not statistically significant(P>0.05).The 50% patients alleviated after treatment, and discharged.Ten cases(one case lose to follow-up) died, which included adult Still disease in 5 cases, lymphoma in 1 case, dermatomyositis in 1 case, vasculitis related to anti-neutrophil cytoplasmic antibody in 1 case, systemic lupus erythematosus in 1 case and mesenteric lipid membrane inflammation in 1 case, and the respiratory failure was the death cause.ConclusionsThe adult still disease in RHS caused by rheumatic disease is often found, the lymphoma is more, poor prognosis and high death rate, and the main death cause of which is respiratory failure.The related indicator test can help to early diagnose the RHS.Hormones combined with cyclophosphamide can control the progression of disease.