Congenital thrombotic thrombocytopenic purpura due to ADAMTS-13 deficiency

Preferred Label : Congenital thrombotic thrombocytopenic purpura due to ADAMTS-13 deficiency;

ICD-11 definition : This is a rare disorder of the blood-coagulation system, causing extensive microscopic clots to form in the small blood vessels throughout the body, due to ADAMTS-13 deficiency.;

ICD-11 synonym : Upshaw-Schulman syndrome;

Details

Origin ID

258428739;

Automatic exact mappings (from CISMeF team)
- Congenital thrombotic thrombocytopenic purpura [ORDO Disease]
- Thrombotic thrombocytopenic purpura, hereditary [OMIM Phenotype]
- Upshaw-Schulman syndrome (disorder) [SNOMED CT concept]
- congenital thrombotic thrombocytopenic purpura [MeSH concept]
- purpura, thrombotic thrombocytopenic [MeSH Descriptor]

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