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

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Gioacchino Li Cavoli https://orcid.org/0000-0002-8252-6588
Francesca Finazzo
Carmela Zagarrigo
Camillo Carollo
Tancredi V. Li Cavoli
Onofrio Schillaci

Keywords

Kasabach-Merrit Syndrome, Systemic angiomatosis, Hemodialysis

Abstract

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.  The authors report the 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, the authors identified a subacute low-grade consumption coagulopathy.

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