不同病因伴发严重噬血细胞综合征9例诊疗体会

目的:探讨噬血细胞综合征(HPS)的临床表现、实验室特点、治疗及预后。方法:回顾性分析9例HPS患者的临床资料。结果:9例HPS患者中有1例为原发HPS,1例感染相关HPS,4例恶性淋巴瘤相关HPS,1例EB病毒HPS,2例病因不明。临床上均有不同程度发热,血细胞不同程度下降,凝血功能异常及铁蛋白升高,其中淋巴结肿大5例,脾大5例。8例治疗患者初期评估有效,目前3例已死亡。结论:HPS临床表现多样,疾病快速进展,须提高对该疾病认识,尽早启动治疗方案,改善预后。

Diagnosis and treatment experience of 9 cases of severe hemophagocytic syndrome accompanied by different etiology

Objective: To investigate the clinical and laboratory features, treatment and prognosis of patients with B cell lymphoma associated hemophagocytic syndrome (HPS). Methods: The clinical data of 9 HPS patients were analyzed retrospectively. Results: Among the 9 patients with HPS, there was one case of primary HLH, one case of infection related HPS, 4 cases of malignant lymphoma related HPS, 1 case of EB virus infection related HPS, and 2 cases of unknown etiology. Clinically, there was fever of different degrees, decreased blood cells to varying degrees, coagulation dysfunction and increased ferritin, including 5 cases of lymphadenopathy and 5 cases of splenomegaly. The treatment was initially effective in 8 cases, and there were 3 deaths at present. Conclusion: The clinical manifestations of HPS are various and the disease progresses rapidly. Enhancing the understanding of the disease and initiating the treatment scheme as soon as possible can improve the prognosis of HPS.