Thrombotic thrombocytopenic purpura
ORPHA:54057· ICD-10 M31.1
Definition
An aggressive and life-threatening form of thrombotic microangiopathy (TMA) characterized by profound peripheral thrombocytopenia, microangiopathic hemolytic anemia (MAHA) and organ failure of variable severity and is comprised of a congenital (cTTP) and acquired, immune-mediated (iTTP) form.
- Prevalence
- Unknown
- Inheritance
- Autosomal recessive, Multigenic/multifactorial
- Age of onset
- All ages