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J Cutan Med Surg. 2009 May-Jun;13(3):155-9.

Vincristine and corticosteroids as first-line treatment of Kasabach-Merritt syndrome in kaposiform hemangioendothelioma.

Author information

1
Department of Paediatrics and Paediatric Dermatology, The Hospital for Sick Children, University of Toronto, Toronto, ON. 5amd2@queensu.ca

Abstract

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.

PMID:
19426625
DOI:
10.2310/7750.2008.08006
[Indexed for MEDLINE]

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