Hemangioma thrombocytopenia syndrome

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Disease Overview

Hemangioma thrombocytopenia syndrome is characterized by profound thrombocytopenia in association with two rare vascular tumors: kaposiform hemangioendotheliomas and tufted angiomas. The profound thrombocytopenia can cause life threatening bleeding and progress to a disseminated coagulopathy in patients with these tumors.[2616][2617] The condition typically occurs in early infancy or childhood, although prenatal cases (diagnosed with the aid of ultrasonography), newborn presentations, and rare adult cases have been reported.[2618]


Synonyms

  • Kasabach Merritt syndrome
  • Thrombocytopenia-hemangioma syndrome
  • Kasabach Merritt phenomenon
  • KMP

For more information, visit GARD.

National Organization for Rare Disorders