The Kasabach-Merrit Syndrome in Patient with Systemic Angiomatosis on Long-Term Hemodialysis: Kasbach-Merrit Syndrome and Systemic Angiomatosis

The Kasabach-Merritt Syndrome, a clotting disorder with consumptive thrombocytopenia and anemia, is associated with vascular lesions. The cardinal features of Kasabach-Merritt Syndrome include a large hemangioma, thrombocytopenia, anemia, hypofibrinogenemia and a prolonged prothrombin time. Kasabach-Merritt Syndrome without visceral hemangiomas is uncommon in adult subjects. We report our experience on adult post-traumatic Kasabach-Merritt Syndrome in a 36-year-old subject on long-term hemodialysis with systemic angiomatosis. The primary pathophysiologic event of the Kasabach-Merritt syndrome is platelet trapping within the vascular lesion. In this experience, we identified a subacute low-grade consumption coagulopathy.