Refractory Kasabach–Merritt phenomenon successfully treated with sirolimus, and a mini‐review of the published work

Kasabach–Merritt phenomenon ( KMP ) is a rare and life‐threatening disease involving a vascular tumor combined with severe consumptive coagulopathy. We present for the first time a case of KMP with the vascular tumor involving two anatomical sites; the patient failed to respond to steroids and vincristine. Following sirolimus therapy at a dose of 0.8 mg/m 2 twice daily, the lesions shrank and the platelet count improved and remained normal 4 months after initial therapy. Current treatments for KMP are not particularly effective. Sirolimus at 0.8 mg/m 2 per dose, administrated twice daily, appears to be a safe and effective management option. It appears to be an interesting therapeutic option in refractory KMP , but the time to response is variable.