LONG-TERM FOLLOW-UP OF A CASE OF KASABACH-MERRITT SYNDROME SUCCESSFULLY TREATED WITH RADIOTHERAPY AND CORTICOSTEROIDS

Kasabach-Merritt syndrome is the association of thrombocytopaenia, spontaneous bleeding, and enlargement of a haemangioma. It is caused by an intense, self-perpetuating process of clot formation and lysis within the abnormal vascular channels of the haemangioma, and results in consumption of platelets and clotting factors. Treatment involves ablation of the lesion with or without pharmacological manipulation of the coagulation and fibrinolytic systems. No single therapeutic modality is universally successful but a combination of radiotherapy and corticosteroids can result in a dramatic, immediate response with minimal long term complications.