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Kasabach-Merritt syndrome

Disease definition

Kasabach-Merritt syndrome (KMS), also known as hemangioma-thrombocytopenia syndrome, is a rare disorder characterized by profound thrombocytopenia, microangiopathic hemolytic anemia, and subsequent consumptive coagulopathy in association with vascular tumors, particularly kaposiform hemangioendothelioma or tufted angioma.

ORPHA:2330

Classification level: Disorder
  • Synonym(s):
    • Hemangioma-thrombocytopenia syndrome
  • Prevalence: Unknown
  • Inheritance: Not applicable 
  • Age of onset: Infancy, Neonatal
  • ICD-10: D18.0
  • OMIM: 141000
  • UMLS: C0221025
  • MeSH: -
  • GARD: 70
  • MedDRA: 10058423

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