Tanja Frevel1, Heike Rabe, Frank Uckert, Erik Harms. 1. Department of Paediatrics, University Children's Hospital Münster, Albert-Schweitzer-Strasse 33, 48149 Münster, Germany. Frevel@uni-muenster.de
Abstract
UNLABELLED: We report the case of a 4-month-old boy presenting with a giant cutaneous haemangioma complicated by Kasabach-Merritt syndrome (KMS) with severe thrombocytopenia. After poor response to corticosteroid therapy and subsequent treatment with interferon alpha-2a, radiotherapy led to tumour regression and resolution of the disseminated intravascular coagulopathy over a 14-month period of follow up. Whereas the various available treatment options are reviewed and discussed in this article, the therapy of choice should be chosen individually. CONCLUSION: to date prospective randomised and controlled trials are required to investigate the optimal management of patients with Kasabach-Merritt syndrome.
UNLABELLED: We report the case of a 4-month-old boy presenting with a giant cutaneous haemangioma complicated by Kasabach-Merritt syndrome (KMS) with severe thrombocytopenia. After poor response to corticosteroid therapy and subsequent treatment with interferon alpha-2a, radiotherapy led to tumour regression and resolution of the disseminated intravascular coagulopathy over a 14-month period of follow up. Whereas the various available treatment options are reviewed and discussed in this article, the therapy of choice should be chosen individually. CONCLUSION: to date prospective randomised and controlled trials are required to investigate the optimal management of patients with Kasabach-Merritt syndrome.