围产期噬血细胞综合征临床诊疗分析

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. CONCLUSION: 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.