British Journal of Dermatology
British Journal of Dermatology
5 years ago

Sirolimus effects on Kasabach-Merritt phenomenon coagulopathy

C Bodemer, E Puzenat, T Leblanc, S Hadj-Rabia, S Proust, D Lasne, O. Boccara
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.

Publisher URL: http://onlinelibrary.wiley.com/resolve/doi

DOI: 10.1111/bjd.15883

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