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.