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.