Clinical analysis of 17 cases of neonatal Kasabach-Merritt syndrome / 国际儿科学杂志

ABSTRACT

Objective To summarize the clinical characteristics and therapeutic approaches of Kasabach-Merritt syndrome (KMS).Methods The data from 17 KMS patients that had been definitely diagnosed from December,2007 to January,2012 in our tertiary center were collected retrospectively.Results We described 17 patients,13 of whom were male and 4 of whom were female,with an age range of 17 hours to 28 days.Of the 17 cases,4 cases had hemangioma of internal organs,13 on the surface of the body.All of them had thrombocytopenia and coagulation dysfunction.Seventeen cases were initially treated with corticosteroids.The responses were variedexcellent and rapid improvement (n =6),failure (n =11),recurrence (n =3).Then the cases of failure and recurrence accepted artery embolizationexcellent and rapid improvement (n =8),failure (n =4),recurrence (n =1),with 1 giving up.At last,the cases resistant to artery embolization therapy accepted vincristine therapy.Four cases had rapid improvement,and 1 died from disseminated intravascular coagulation.In this study,a response rate to corticosteroids was 35.3％,and the recurrent rate was 50％.The response rate to artery embolization was 61.5％.Five patients unresponsive to hormone therapy and artery embolism were treated with vincristine,and the effective rate was 80％.Conclusion In the therapy of neonatal KMS,the resistant to corticosteroids is common.Combinative therapy of corticosteroids with artery embolization is recommended as the first-line therapy.Vincristine is suggested as a therapy when there is resistance to the other therapy.