Kasabach–Merritt syndrome: a review of the therapeutic options and a case report of successful treatment with radiotherapy and interferon alpha

Abstract

We describe the successful treatment of a neonate with Kasabach-Merritt syndrome who received local irradiation and interferon alpha therapy after failure of corticosteroid treatment. A male neonate, born after an uneventful pregnancy, had a huge haemangioma involving the upper right cervical region as well as severe thrombocytopenia. He was treated with corticosteroids, interferon alpha and radiotherapy. Prednisolone therapy (5 mg kg(-1) day(-1)) was started at 41 days of age. No therapeutic effect was observed after 2 weeks. At this time the tumour size had increased dramatically, platelet counts had decreased progressively and coagulation abnormalities had developed. Because corticosteroid therapy had been ineffective and the child was in a life-threatening condition, irradiation was delivered up to a total dose of 9.5 Gy in five fractions. Simultaneously, prednisolone therapy was slowly decreased and interferon alpha therapy (3 million U m(-2) day(-1)) was started and continued for 6 weeks. After irradiation with 9.5 Gy and beginning interferon alpha therapy, the tumour decreased in size and coagulation parameters normalized within 4 weeks. 6 months later, platelet counts and coagulation parameters were still normal. The tumour had further decreased in size. No acute severe side effects were observed. Radiation therapy combined with interferon alpha treatment is an alternative treatment modality when high dose corticoid steroid therapy has been ineffective in patients with Kasabach-Merritt syndrome, despite the risks of growth delay and secondary malignancy. In children showing no response to corticosteroids, radiotherapy and/or interferon alpha should be considered in Kasabach-Merritt syndrome.

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

“…Historically, radiation therapy has been associated with multiple long-term side effects when used in pediatric population [60,100,[113][114][115][116] its use in adults has been more liberal. In a series of 28 patients, concurrent radiation and steroids demonstrated a 75% response rate, with no radiation-induced side effects over a median follow-up of more than 6 years [117].…”

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

“…However, treatment with these regimens can result in severe adverse events. The known adverse effects of interferon therapy include flu-like symptoms, neutropenia, and spastic diplegia in infancy 5,7 . Vincristine can be associated with transient peripheral neuropathy, and it is considered a relatively safe regimen with high efficacy 7,8,15 .…”

“…Vincristine can be associated with transient peripheral neuropathy, and it is considered a relatively safe regimen with high efficacy 7,8,15 . Since radiotherapy has been reported to induce late side effects, including local growth failure, late skin changes, destruction of bone tissue and secondary malignancies, it should be administered to patients with life-threatening lesions, or to patients with lesions that may compromise organ function, or when other therapies fail to achieve response [5][6][7]15 .…”

“…† It seemed to achieve high rate of initial response as well as short duration of therapy 18 . ‡ It should be considered for patients with life-threatening lesions or with lesions that might compromise organ function or if other therapies fail to achieve a response 5,15 . Our case: Intravenous dexamethasone 0.32 mg/kg/day (equivalent to prednisolone 2 mg/kg/day) for 2 days, gradually tapered to oral prednisolone at a dose of 1.5, 1.2, 1.0 over 15 days, and 0.5 mg/kg/day over two weeks.…”

“…With an overall mortality rate of 12%, various treatment regimens have been introduced to manage KMS 5 . Recent reports recommended a careful stepwise approach in the management of KMS, but the results demonstrated inconsistent efficacy 6,7 .…”