Treatment of Kasabach-Merritt syndrome: a stepwise regimen of prednisolone, dipyridamole, and interferon
- 1 September 2003
- journal article
- research article
- Published by Wiley in International Journal of Dermatology
- Vol. 42 (9), 741-748
- https://doi.org/10.1046/j.1365-4362.2003.01796.x
Abstract
Background 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. Methods All patients were first treated with oral corticosteroid (initially 3 mg/kg/day then 5 mg/kg/day) and dipyridamole. Interferon‐alpha‐2b (IFNα2b) 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. Results 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, IFNα2b successfully induced regression of the tumor and increased the platelet count in six patients; however, IFNα2b was successful as monotherapy in only three cases; two patients died of aspiration pneumonia whilst on therapy and one patient could not be taken off IFNα2b until weekly vincristine was given (eight doses). Two other patients did not respond to IFNα2b in 4 weeks; embolization and vinblastine was used in one patient to induce regression of the tumor and resolution of thrombocytopenia. Hypertension developed in all children on high‐dose prednisolone. Conclusions KMS may be treated in a stepwise approach. High‐dose steroid does not result in a high response rate and is not tolerated well. The response to IFNα2b is more favorable, but life‐threatening adverse events may occur. Chemotherapy with vincristine or vinblastine may be useful as adjunctive therapy in KMS, but experience is still limited.Keywords
This publication has 23 references indexed in Scilit:
- Effective therapy of a vascular tumor of infancy with vincristineJournal of Pediatric Surgery, 2001
- Early surgical intervention in a patient with Kasabach-Merritt phenomenonThe Journal of Pediatrics, 2001
- Residual lesions after Kasabach-Merritt phenomenon in 41 patientsJournal of the American Academy of Dermatology, 2000
- Infants with Kasabach-Merritt syndrome do not have “true” hemangiomasThe Journal of Pediatrics, 1997
- Kaposiform haemangioendothelioma associated with Kasabach‐Merritt syndromeHistopathology, 1996
- Kasabach-Merritt coagulopathy complicating Klippel-Trenaunay-Weber syndrome in pregnancyObstetrics & Gynecology, 1995
- Kaposiform Hemangioendothelioma of Infancy and ChildhoodThe American Journal of Surgical Pathology, 1993
- Osteomyelitis Due to Pseudallescheria boydiiSouthern Medical Journal, 1993
- Interferon Alfa-2a Therapy for Life-Threatening Hemangiomas of InfancyThe New England Journal of Medicine, 1992
- CAPILLARY HEMANGIOMA WITH EXTENSIVE PURPURAAmerican Journal of Diseases of Children, 1940