mohammed mokhtar bekkar

Abstract

Introduction: Kasabach-Merritt syndrome is a rare form of vascular tumor that are often misdiagnosed, which is complicated by intra-tumor coagulopathy and requiring urgent management.

Objective: This review highlights the clinical presentation, histopathology, management, and treatment of Kasabach-Merritt syndrome. A clinical case compiled in the pediatric department of the university Hospital Center of Oran Algeria, is described to illustrate the presentation and our management of a patient with Kasabach-Merritt syndrome.

Observation: A female infant was admitted, on the thirtieth day of life, for a purplish red swelling of the left hemiface. He had hemolytic anemia, profound thrombocytopenia and consumptive coagulopathy. It was successfully managed with a combination of high dose of oral corticosteroids and vincristine.

Conclusion: Vincristine and oral corticosteroids were useful for our patient and may be considered an option for first-line therapy of Kasabach-Merritt syndrome. The absence of a codified therapeutic protocol makes the management of this tumor a real challenge.

Keywords: Kasabach-Merritt Syndrome; Thrombocytopenia; Hemangiomas; Coagulopathy; Vascular Tumor; Kaposiform Hemangioendothelioma