嗜血细胞综合征四例分析并文献复习

A Report of 4 Cases and Literatures Review of Hemophagocytic Syndrome

目的分析嗜血细胞综合征(hemophagocytic syndrome,HPS)的临床特征、诊断及治疗,减少误诊误治。方法回顾性分析我院收治的4例HPS的临床资料,并复习相关文献。结果本组均以发热为主要表现,1例收治后即明确诊断;3例病初误诊为上呼吸道感染并白细胞减少症。4例均有发热、高脂血症、凝血功能异常、血细胞减少及血清铁蛋白异常增高等症状,后行骨髓穿刺细胞学检查,3例出现组织嗜血细胞,确诊为HPS;1例依据临床表现及医技检查确诊为HPS。予糖皮质激素、抗感染、免疫抑制药物等治疗,症状未见缓解,均因多脏器功能衰竭死亡。结论HPS临床表现多样,病情进展迅速,若遇及不明原因发热超过1周、抗感染治疗效果差的患者,应考虑到HPS的可能,尽早行相关检查,减少误诊误治。

Objective To analyze clinical features, diagnosis and treatment of hemophagocytic syndrome （HPS） in order to avoid misdiagnosis and mistreatment. Methods Clinical data of 4 patients with HPS was retrospectively analyzed, and related literature was also reviewed. Results The main manifestation of the 4 patients was fever. Among them, one was confirmed the diagnosis immediately after hospitalization; other three patients were misdiagnosed as having upper respiratory tract infection combined with leucopenia, and symptoms such as fever, hyperlipidemia, coagulation disorders, thrombocytope-nia and abnormally elevated serum ferroprotein were all found in four patients. Bone marrow aspiration of cytological examina- tion showed that three patients had hemophagocytic ceils, and HPS was confirmed; one was confirmed as having HPS by clini- cal manifestations and laboratory examinations. Symptomatic treatments such as glucocorticoid, anti-infection and immunosup- pressive therapy were given, but the symptoms were not relieved, and then all patients died of muhiorgan failure. Conclusion Clinical manifestations of HSP is diversified with rapid development. Clinicians should consider the possibility of HSP for patients having fever of undetermined origin for more than 1 week and poor effect of anti-infection therapy, and give related ex- aminations as early as possible to avoid misdiagnosis and mistreatment.