Congenital thrombotic thrombocytopenic purpura
ORPHA:93583· ICD-10 M31.1
Definition
A hereditary form of thrombotic thrombocytopenic purpura (TTP) characterized by profound peripheral thrombocytopenia, microangiopathic hemolytic anemia (MAHA) and single or multiple organ failure of variable severity.
- Prevalence
- <1 / 1 000 000
- Inheritance
- Autosomal recessive
- Age of onset
- Adult, Childhood, Infancy, Neonatal