The effects of sirolimus on Kasabach–Merritt phenomenon coagulopathy

Kasabach-Merritt phenomenon (KMP) is a very rare life-threatening condition that combines a vascular tumor belonging to the kaposiform hemangioendothelioma (KHE) spectrum lesions, and thrombocytopenia.1,2 Thrombocytopenia results from platelet trapping within the tumor. Platelet activation leads to various degrees of decreased fibrinogen and elevated D-dimer levels. This coagulopathy is frequently protracted after thrombocytopenia resolution. Several treatments have been proposed with variable efficiency. This article is protected by copyright. All rights reserved.