Donjeta Bali Alia, Anila Godo, Emarjola Brahimllari, Mirela Xhafa, Arber Arapi, Leart Berdica, Alma Barbullushi
Abstract: Aims To demonstrate the efficacy of treatment with propranolol and vincristine in a case with kaposiform hemangioendothelioma and Kasabach-Merrit syndrome (KMS) Materials and Methods This is a retrospective case report of a 5 month old girl admitted to our hospital because of a progressive purple, indurated large cutaneous lesion on her right upper anterior-posterior thoracic portion which extends toward neck. Laboratory evaluations showed thrombocytopenia, coagulopathy with a low fibrinogen and a high D-dimer plasma level. The clinical and laboratory investigations suggested the diagnosis of KHE with KMP. We started prednisone 4mg/kg/d and propranolol 1mg/kg/d and thereafter vincristine 0.05 mg/kg/P, once a week. Results A dramatically clinical decrease of the lesion was seen during treatment with prednisone and propranolol but with very low platelet count and massive bleeding.Treatment with vincristine achieved a complete normalization of the platelet count. Neither regrowth of the tumor has been observed until now. Conclusion Propranolol and vincristine seems to be an important addition for the life-threatening condition of KHE with KMS. We concluded that effect of propranolol was solely on the KHE and vincristine showed extraordinary effect on KMS, which need to be communicated in the treatment for future patients.
Keywords: KHE-Kaposiform Hemangioendotelioma, KMS- Kasabach-Merritt Syndrome, prednisone, propranolol, vincristine