噬血细胞综合征8例报道及文献复习

Hemophagocytic syndrome:A report of 8 cases and review of literature

目的:探讨噬血细胞综合征(hemophagocytic syndrom e,HPS)的诊断和治疗。方法:对8例HPS患儿的临床特征、实验室资料、诊治过程进行分析,并结合文献进行讨论。结果:8例HPS患儿均有不明原因持续发热、肝脾肿大(其中脾肿大5例),外周血象两系血细胞减少3例,全血细胞减少5例;骨髓细胞学检查均见网状吞噬细胞增生,噬血细胞易见。1例确定为由巨细胞病毒(CMV)感染所致,治疗后好转。结论:HPS属少见病,临床表现复杂,对于出现上述临床表现的病例应及时或多次的骨髓细胞学检查,可提高诊断率,为进一步的治疗研究创造条件。

Objective： To explore the diagnosis and treatment of hemophagocytic syndrome （HPS）. Methods： The clinical manifestations,laboratory findings, diagnosis and treatment of 8 cases of HPS were analyzed and reviewed with current literature. Results ： Prolonged fever with unknown origin and hepatosplenomegaly were present in all the 8 cases （ splenomegaly in 5 cases ）. Cytopenia with bilineage in the peripheral blood was found in 3 cases and pancytopenia in 5 cases. Hemophagocytosis in bone marrow was easily observed. The only patient infected by cytomegalovirus recovered rapidly after effective treatment. Conclusions ： HPS is a rare disease with complex clinical presentation. Bone marrow aspiration should be performed in time or repeatedly to increase the diagnosis rate, which is of great value to further treatment.