KASABACH-MERRITT SYNDROME

Course overview

KASABACH-MERRITT PHENOMENON (KMP), FIRST DESCRIBED IN 1940, IS A RARE BUT LIFE-THREATENING COAGULOPATHY OF INFANCY WHICH PRESENTS WITH THROMBOCYTOPENIA, MICROANGIOPATHIC HEMOLYTIC ANEMIA, AND CONSUMPTIVE COAGULOPATHY IN THE SETTING OF A RAPIDLY ENLARGING VASCULAR TUMOR. IT IS EXCLUSIVELY ASSOCIATED WITH THE VASCULAR TUMORS KAPOSIFORM HEMANGIOENDOTHELIOMA (KHE) AND TUFTED ANGIOMA (TA), WHICH EXIST ALONG THE SAME NEOPLASTIC SPECTRUM. TREATMENT INCLUDES SUPPORTIVE THERAPY AND MANAGEMENT OF THE UNDERLYING TUMOR. THIS ACTIVITY REVIEWS THE EVALUATION AND TREATMENT OF KASABACH MERRITT SYNDROME AND ADDRESSES THE ROLE OF THE INTERPROFESSIONAL TEAM IN MANAGING THIS RARE CONDITION.