Wednesday February 17, 2010


Q; What is Upshaw-Schülman syndrome (USS)?

Answer: A hereditary form of TTP (thrombotic thrombocytopenic purpura) is called the Upshaw-Schülman syndrome. Patients with this condition develop TTP in clinical situations with increased von Willebrand factor levels, e.g. infection. About 5-10% of all TTP cases are due to Upshaw-Schülman syndrome.

Patients with Upshaw-Schulman syndrome (USS) have repeated episodes of thrombocytopenia and microangiopathic hemolytic anemia that respond to infusions of fresh frozen plasma. Inheritance of USS has been thought to be autosomal recessive, because 2 siblings in the same family are often affected but their parents are asymptomatic.