4.5 Article

Case report: Experience of a rare case of rebound of the Kasabach-Merritt phenomenon during sirolimus treatment in kaposiform hemangioendothelioma

Journal

FRONTIERS IN PEDIATRICS
Volume 10, Issue -, Pages -

Publisher

FRONTIERS MEDIA SA
DOI: 10.3389/fped.2022.949950

Keywords

kaposiform hemangioendothelioma; Kasabach-Merritt phenomenon; embolization; ablation; vincristine; prognosis

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Kaposiform hemangioendothelioma (KHE) is a rare vascular neoplasm associated with life-threatening coagulopathy. Standard treatment protocols for KHE with associated Kasabach-Merritt phenomenon (KMP) are limited. We present a rare case of rebound KHE/KMP during systemic sirolimus treatment, successfully managed with embolization, microwave ablation, and vincristine infusion.
Kaposiform hemangioendothelioma (KHE) is a rare vascular neoplasm associated with the Kasabach-Merritt phenomenon (KMP), which is a consumptive coagulopathy with associated potentially life-threatening thrombocytopenia. There are no standardized treatment protocols for the management of KHE with KMP. Moreover, there are limited reports regarding the treatment of cases of rebound. Herein, we describe a rare case of rebound of KHE/KMP, during systemic sirolimus treatment, successfully treated with embolization and vincristine infusion combined with microwave ablation.

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