Kaposiform Hemangioendothelioma Complicated by a Kasabach-Merritt Syndrome: Report of a Case

Abstract

Kaposiform hemangioendothelioma (KHE) is a very rare form of vascular tumor, which is complicated by intra-tumor coagulopathy and requiring urgent management. 

Objective: we report a case of a KHE, complicated by a kasabach merrit syndrome compiled in the neonatology department of Mohamed VI university hospital, Marrakech and we discuss the different therapeutic modalities.  Observation: a newborn was admitted, on the third day of life, for jaundice associated with a purplish red swelling of the left hemi face. He had anemia and thrombocytopenia requiring repeated transfusions. In view of the instrumental delivery, a hematoma was initially evoked. But in the absence of improvement, the eventuality of a hemangioma or complicated tumor became more likely. The patient was treated by propranolol in addition to high dose of oral corticosteroids and a skin biopsy was realized. Despite treatment, the patient died 10 days later due to a disseminated intravascular coagulation. A posteriori the histological study confirmed the KHE.  Conclusion: a codified and early therapeutic protocol is necessary to improve the prognosis of patients with KEH, especially since other therapeutics has proved their effectiveness in the literature.