Treatment of refractory Kasabach-Merritt syndrome with transcatheter arterial embolization plus sirolimus therapy / 中华放射学杂志

ABSTRACT

Objective To investigate the efficacy and safety of transcather arterial embolization (TAE) plus sirolimus for the treatment of refractory Kasabach-Merritt syndrome (KMS) in infants. Methods Clinical data of twelve infants with refractory KMS treated between December 2015 and October 2016 in a single hospital were retrospectively analyzed. TAE were performed in all patients after failed traditional multiple therapies, followed by oral sirolimus administration. The dose of sirolimus was modulated according to the level of sirolimus, the count of platelet, the shrinkage of the lesion and the side effects, which were monitored regularly during the study. Results All 12 patients were treated with TAE plus sirolimus therapies successfully. The platelet count for all patients increased to≥100×109/L for the first time at (7±5) days. Stabilization of platelet level was obtained in (15±7) days averagely. Before the treatment, two infants had a normal fibrinogen level and the fibrinogen level in the other 10 infants was found to be increased to≥2.0 g/L at (9 ± 4)days for the first time and was then stabilized at levels>2.0 g/L at (19 ± 7)days after treatment. One patient showed skin fester (GradeⅡ), one patient had a fever with acute pulmonary infection (Grade Ⅲ) and both patients improved well after symptomatic treatment. There were no serious complications in the other ten patients. Conclusions TAE plus sirolimus can rapidly improve levels of platelets and fibrinogen, and it is a safe, useful and effective method for treatment of refractory KMS in infants.