Vincristine for the treatment of Kasabach-Merritt syndrome: recent New Zealand case experience.
Department of Radiology, Christchurch Public Hospital, Christchurch. Kimberley.Thomson@cdhb.govt.nz
AIMS: To present a case series showing efficacious use of vincristine in treating Kasabach-Merritt syndrome (KMS).
METHODS: The case notes of four children treated for KMS by the authors with corticosteroids and vincristine were reviewed. Specific attention was paid to the efficacy and adverse effects of each therapeutic agent.
RESULTS: The age of presentation ranged from birth to 11 months. Initial treatment with high dose corticosteroids was uniformly ineffective, and in 2 cases, prolonged use caused significant side-effects. Subsequent or concurrent treatment with vincristine was effective and well-tolerated, with no discernable side effects. The only complications were line-related.
CONCLUSIONS: Kasabach-Merritt syndrome is rare, but it is associated with significant morbidity and mortality. No definitive treatment regime has been established, but the authors suggest that vincristine should be considered a first-line agent, and that the use of systemic corticosteroids should not be routine.
PMID: 17308555 [PubMed - indexed for MEDLINE]