噬血细胞综合征8例报道及文献复习

目的: 探讨噬血细胞综合征(hemophagocytic syndrome,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。

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

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.