[Upshaw-Schulman syndrome]

Upshaw-Schulman syndrome is a rare congenital disease, with thrombocytopenia and microangiopathic hemolytic anemia, which can be temporarily and repeatedly corrected by the transfusions of plasma from normal donors. Although the pathogenesis of this congenital disorder remains unknown, it appears that patients with this syndrome lack an unidentified plasma factor, which is normally present in plasma and is genetically determined.