Vincristine and Corticosteroids as First-Line Treatment of Kasabach-Merritt Syndrome in Kaposiform Hemangioendothelioma

Background: Historically, patients with the consumptive coagulopathy Kasabach-Merritt syndrome (KMS) have been treated with systemic corticosteroids as first-line therapy, but many patients do not respond. Recently, there have been increasing reports of the use of the chemotherapeutic drug vincristine in these patients. Objective: To report a case of a newborn with a kaposiform hemangioendothelioma (KHE) of the right leg associated with KMS treated successfully with vincristine and oral corticosteroids. Methods: The patient's chart and the literature on the subject were reviewed using Medline and PubMed. Results: Treatment with vincristine and corticosteroids lead to sustained shrinking of the tumor and correction of the thrombocytopenia and coagulopathy through 1 year of age. We believe this is the first report in the North American literature of corticosteroids and vincristine being used concomitantly as first-line therapy for KHE with KMS. Conclusion: Vincristine and corticosteroids should be considered first-line treatment for KMS.