Radiotherapy for Kasabach-Merritt (KM) phenomenon in an adult patient with benign vascular neoplasm- a case report

Abstract

Introduction: Kasabach Merritt (KM) phenomenon is consumption coagulopathy in a vascular neoplasm. The combination of haemangioma, thrombocytopenia, and coagulopathy is termed Kasabach-Merritt phenomenon. Vascular neoplasms are more common in children wherein they may resolve spontaneously. Kaposi form hemangioendothelioma and tufted angioma are most common vascular neoplasms in children associated with Kassabah-Merritt phenomenon but are found with other vascular neoplasms too. These lesions are rare in adults. Radiotherapy was the treatment of choice in the past in children but not considered in the absence of life threatening complication presently due to its late effects. The other modalities that are tried are surgery, embolization, laser surgery, and pharmacologic agents such as steroids, interferons, and cyclophosphamide. We report a persistent 10 years’ long response of a benign vascular neoplasm with Kasabach-Merritt phenomenon and life threatening bleeding to radiotherapy in an adult with asymptomatic late side effects. Case report: A 35-year-old lady from south India presented with a 15x15 cm left lower back swelling and hemorrhagic pleural effusion and thrombocytopenia. She received radiotherapy of 40 Gy in 20 fractions to the swelling. Swelling, effusion and thrombocytopenia resolved and remain resolved for 10 long years. She did have asymptomatic late side effects of radiotherapy. Conclusion: Though radiation therapy is not considered in children with KM phenomenon, it could be considered as an option of treatment in adults with KM phenomenon.