Abstract: Xiao Yuanyuan, Xu Zigang, Xu Zhe, Xiang Xin, Liu Ying, Ma Lin, Yang Zhou Department of Dermatology, Beijing Children′s Hospital, Capital Medical University, National Center for Children′s Health, Beijing 100045, China Corresponding author: Xu Zigang, Email: zigangxu@yahoo.com 【Abstract】 Objective To explore effective therapy for Kasabach-Merritt syndrome （KMS）. Methods Clinical data were collected from 11 infants with KMS in the Department of Dermatology, Beijing Children′s Hospital affiliated to Capital Medical University between 2015 and 2017, and the efficacy of vincristine combined with glucocorticoids for the treatment of KMS was evaluated. Results Of the 11 patients, 4 were male and 7 were female. Their average age at the first clinic visit was 87.91 ± 72.01 days （range, 1 - 212 days）. Most of the hemangiomas manifested as hard violaceous plaques, and skin purpura occurred around the hemangiomas in 5 patients. The initial blood platelet counts of the 11 patients ranged from 4 × 109/L to 32 × 109/L. All the patients were treated with oral prednisone at doses of （2 - 5） mg·kg-1·d-1 combined with intravenous injection of vincristine at a dose of 0.05 mg/kg every week. Among 10 patients treated with glucocorticoids and vincristine, the blood platelet counts became normal after （1.8 ± 1.23） weeks of treatment, the fibrinogen levels returned to normal after （3.6 ± 1.26） weeks of treatment, and the hemangiomas started to become softer and smaller after （3.9 ± 0.74） weeks. The blood platelet count still did not return to normal in 1 patient after 5 weeks of vincristine treatment. After the treatment with venous embolization, the blood platelet count gradually became normal and remained stable. Conclusion Vincristine combined with glucocorticoids can control the development of hemangioma in infants with KMS, and improve the recovery of blood platelet count.

Key words: Hemangioma, Thrombocytopenia, Vincristine, Glucocorticoids, Treatment outcome