围产期噬血细胞综合征临床诊疗分析

Clinical diagnosis and treatment of perinatal stage hemophagocytic lymphohistiocytosis

目的分析围产期相关噬血细胞综合征(hemophagocytic lymphohistiocytosis,HLH)的临床特征.方法对2011年1月至2016年10月诊治的11例围产期HLH患者基本信息、临床特点、相关疾病/因素和治疗效果进行观察.结果11例HLH患者中,初产妇9例.妊娠期发病5例,产后发病6例.6例患者存在相关疾病/因素,感染最为常见(6例).治疗方案包括糖皮质激素+氟达拉滨1例,HLH-94/04方案9例,单药激素1例.最终2例患者死亡.结论围产期(妊娠28周至产后7 d)HLH在妊娠/产后相关HLH噬血中很常见.感染是最常见的相关因素.终止妊娠后进行治疗是有效的,而仅仅终止妊娠可能还不足以控制围产期相关HLH.围产期相关HLH的转归优于其他继发性HLH亚型.

Objective To analyze the clinical features of patients with hemophagocytic lymphohistiocytosis(HLH)during perinatal stage.Methods From January 2011 to October 2016,the obstetric materials,clinical features,associated disease/factors and therapeutic outcomes were studied in 11 patients with HLH in perinatal period.Results In these 11 patients,9 were primipara.As for the onset time of HLH,5 were before delivery and 6 were after delivery.Six were complicated with other associated disease/factors,and infection was the commonest(5/6).Treatment included glucocorticoid+fludarabine in 1 case,HLH-94/04 protocols in 9 cases,and monotherapy corticosteroids in 1 case.Two patients died eventually.Conclusions Perinatal HLH is commonly observed in the pregnancy/postpartum related HLH(28th week of pregnancy to one week after delivery).Infection is still the commonest associated factors.HLH-94/04 protocols after the cessation of pregnancy may be effective,but the cessation of pregnancy itself may not be enough for the perinatal stage related-HLH.The perinatal stage related-HLH still has a better outcome than the other subtype of secondary-HLH.