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.


CONCLUSIONS
Vincristine and corticosteroids should be considered first-line treatment for KMS.
Department of Paediatrics and Paediatric Dermatology, The Hospital for Sick Children, University of Toronto, Toronto, ON. 5amd2@queensu.ca
Aaron M
AM
Drucker
Elena
E
Pope
Sanjay
S
Mahant
Miriam
M
Weinstein
D000284:Administration, Oral; D000972:Antineoplastic Agents, Phytogenic / Q000008:administration & dosage / Q000627:therapeutic use*; D001706:Biopsy; D001778:Blood Coagulation Disorders / Q000150:complications / Q000188:drug therapy*; D003937:Diagnosis, Differential; D004359:Drug Therapy, Combination; D005260:Female; D005500:Follow-Up Studies; D005938:Glucocorticoids / Q000008:administration & dosage / Q000627:therapeutic use*; D006390:Hemangioendothelioma / Q000150:complications / Q000188:drug therapy* / Q000473:pathology; D006801:Humans; D007231:Infant, Newborn; D012867:Skin / Q000473:pathology*; D013577:Syndrome; D014750:Vincristine / Q000008:administration & dosage / Q000627:therapeutic use*; D014983:Xeroderma Pigmentosum / Q000150:complications / Q000188:drug therapy* / Q000473:pathology
D000972:Antineoplastic Agents, Phytogenic; D005938:Glucocorticoids; D014750:Vincristine