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Kasabach-Merritt syndrome

ORPHA2330
Synonym(s) Hemangioma-thrombocytopenia syndrome
Prevalence Unknown
Inheritance Not applicable
Age of onset Infancy
Neonatal
ICD-10
  • D18.0
OMIM
UMLS
  • C0221025
MeSH -
MedDRA
  • 10058423

Summary

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.

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