Treatment of Kasabach-Merritt syndrome: a stepwise regimen of prednisolone, dipyridamole, and interferon

International Journal of Dermatology
Siriwan WananukulPanya Seksarn

Abstract

Kasabach-Merritt syndrome (KMS) is a rare, aggressive, vascular tumor with thrombocytopenia and consumptive coagulopathy. A standard treatment regimen for KMS has not been established. We reviewed our experience of a stepwise approach for the treatment of 10 children with KMS. All patients were first treated with oral corticosteroid (initially 3 mg/kg/day then 5 mg/kg/day) and dipyridamole. Interferon-alpha-2b (IFNalpha2b) was used as second therapy for steroid-resistant cases for 12 months, then tapered to an alternate-day regimen, and then discontinued. Adjunctive therapy, including embolization of the feeding vessel or chemotherapy, was additionally used in patients who failed to respond to IFN or could not be taken off IFN. Ten patients were treated on this protocol with a follow-up time of 1-6 years. Oral corticosteroid plus dipyridamole was successful in four patients, but was tapered off by 12 months without recurrence in only two cases. Of the eight steroid-resistant cases, IFNalpha2b successfully induced regression of the tumor and increased the platelet count in six patients; however, IFNalpha2b was successful as monotherapy in only three cases; two patients died of aspiration pneumonia whilst on therapy and one patie...Continue Reading

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Citations

Dec 17, 2010·International Journal of Hematology·Yuko WatanabeShigeru Tsuchiya
Nov 3, 2009·Journal of Neurosurgery. Pediatrics·Stacey Quintero WolfeMohammad Ali Aziz-Sultan
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