Sirolimus effects on Kasabach-Merritt phenomenon coagulopathy

Abstract

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.

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