Kaposiform hemangioendothelioma with Kasabach–Merritt phenomenon in a neonate – role of dual therapy: A case report and review of literature

Kaposiform hemangioendothelioma (KHE) represents a rare, borderline vascular tumor with locally aggressive behavior. They are often associated with a potentially life-threatening coagulopathy known as Kasabach–Merritt phenomenon (KMP). Due to heterogeneous nature of the vascular lesion and lack of s...

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Bibliographic Details
Main Authors: Gaurav Parashar, Gowri Shankar, Ravindra Sahadev, Ramesh Santhanakrishnan
Format: Article
Language:English
Published: Wolters Kluwer Medknow Publications 2020-01-01
Series:Journal of Indian Association of Pediatric Surgeons
Subjects:
Online Access:http://www.jiaps.com/article.asp?issn=0971-9261;year=2020;volume=25;issue=3;spage=178;epage=181;aulast=Parashar
Description
Summary:Kaposiform hemangioendothelioma (KHE) represents a rare, borderline vascular tumor with locally aggressive behavior. They are often associated with a potentially life-threatening coagulopathy known as Kasabach–Merritt phenomenon (KMP). Due to heterogeneous nature of the vascular lesion and lack of standardized treatment protocols, these patients pose a diagnostic dilemma and therapeutic challenge with morbidity and potential mortality. We report successful management of an infant with KHE and associated KMP. Difficulties encountered in diagnosis, initiation of therapy, and role of dual therapy with vincristine and steroids are discussed.
ISSN:0971-9261
1998-3891