Stepwise multimodal approach in the treatment of Kasabach-Merritt syndrome

Background: The purpose of the present study was to evalutate the various t reatment modalities for Kasabach-Merritt syndrome (KMS) and to identify the most reliable treatment modalities. Methods: A retrospective analysis was performed on 37 KMS patients who were admitted to Seoul National University Hospital between January 1979 and Ju ne 1999. Age, sex, locations of the hemangiomas, clinical symptoms and hema tologic data were analyzed by multivariate logistical regression analysis t o determine the response to various treatment modalities. Results: Twenty-four of 37 patients (20 boys and 17 girls) were diagnosed d uring infancy. The locations of hemangioma were superficial skin in 31 pati ents, combined skin and visceral organs in four patients and visceral organ s only in two patients. Tumor size was more than 5 cm in diameter in all ca ses, except for one. The treatment principle of KMS in our center involves a stepwise multimodal approach. Of the 37 cases, surgical resection was per formed in two. Steroids were tried initially in 35 patients over a I week p eriod, with an initial response rate of 11.4% (4/35). Combined steroid/radi ation treatment was given in 28 cases, with a response rate of 75.0% (21/28 ). Four of five patients with additional interferon (IFN)-alpha therapy imp roved. No significant correlations were found between the prognostic factor s, such as age, sex, size and locations of the hemangioma, hematologic data and the treatment modalities. Conclusions: Based on the experiences in a single center over a period of 2 0 years, we recommend a stepwise multimodal approach for the treatment of K MS; for example, steroid, radiation therapy and IFN-a in this order. Howeve r, surgical treatment is helpful if total resection is possible.