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

Abstract

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 IFNalpha2b 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.

Clinical Review: Management of Adult Kasabach-Merritt Syndrome Associated with Hemangiomas

“…Rather than concurrent treatment, more number of authors of published literature suggests use of sequential approach with various modalities for KMS [23,24]. The general approach is to manage coagulopathy effectively with supportive care and medical management [23][24][25][26][27][28][29][30] …”

“…The general approach is to manage coagulopathy effectively with supportive care and medical management [23][24][25][26][27][28][29][30] …”

Kasabach-Merritt Syndrome Arising from Tufted Angioma Successfully Treated with Systemic Corticosteroid

“…One study demonstrated that treatment with prednisolone at 2 or 3 mg/kg/day achieved response in 30% to 72% of infants 16 . However, other data suggested variable response to prednisolone, ranging from no efficacy to significant regression in lesion size, and even complete remission 7 . The discrepant results may be due to variation in patient clinical characteristics in the studies, such as different pre-existing tumors, and the possibility that the enrolled patients actually had vascular malformations, rather than hemangiomas.…”

“…Radiotherapy is another option which can induce embolization within the hemangiomas. These options are usually recommended as second-line therapies 6,7,15 . However, treatment with these regimens can result in severe adverse events.…”

“…Recent reports recommended a careful stepwise approach in the management of KMS, but the results demonstrated inconsistent efficacy 6,7 . Among the regimens recommended, systemic corticosteroid is considered as first-line therapy for KMS patients because of its low cost and ease of administration [6][7][8] . We report a case of KMS arising from tufted angioma successfully treated with systemic corticosteroid.…”