Case Report Recognition and Treatment of Early Kasabach-Merritt Phenomenon in Infant with Rare Kaposiform Hemangioendothelioma: A Case Report
by Natalie Mastroianni1, Rita Sico2, Sharda Udassi3*
1Department of Pediatrics, West Virginia University School of Medicine, West Virginia, USA
2Department of Radiology, West Virginia University School of Medicine, West Virginia, USA
3Department of Pediatrics, West Virginia University School of Medicine, West Virginia, USA
*Corresponding author: Sharda Udassi, Department of Pediatrics, West Virginia University School of Medicine, West Virginia, USA.
Received Date: 26 July, 2024
Accepted Date: 07 August, 2024
Published Date: 12 August, 2024
Citation: Mastroianni N, Sico R, Udassi S (2024) Recognition and Treatment of Early Kasabach-Merritt Phenomenon in Infant with Rare Kaposiform Hemangioendothelioma: A Case Report. Clin Exp Dermatol Ther 9: 225. https://doi.org/10.29011/2575-8268.100225
Abstract
Kaposiform hemangioendotheliomas are very rare, locally invasive vascular neoplasms found most commonly during infancy or early childhood. Although rarely of metastatic potential, they carry a high morbidity and mortality rate due in part to the risk of Kasabach-Merritt phenomenon (KMP). KMP is a consumptive coagulopathy characterized by platelet trapping and aggregation within a large vascular malformation. The resulting utilization of coagulation factors can quickly progress into disseminated intravascular coagulation and severe hemorrhage. Due to the extreme rarity of these tumors, standardized treatment guidelines are lacking. Corticosteroids have historically been the first line treatment, yet the vast majority of neoplasms are steroid unresponsive. Described below is a case report of an infant presenting with a locally invasive kaposiform hemangioendothelioma with the early stages of KMP. Treatment with a prednisolone course and sirolimus for an indefinite time was used. More research is needed to determine the optimal therapy and treatment duration of this rare vascular neoplasm.
Keywords: Hemangioendothelioma; Kasabach-Merritt; Coagulopathy; Sirolimus
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