![cover image](https://wikiwandv2-19431.kxcdn.com/_next/image?url=https://upload.wikimedia.org/wikipedia/commons/thumb/2/25/Blood_smear_with_typical_schistocytes_in_TTP_marked_in_blue_1.tif/lossy-page1-640px-Blood_smear_with_typical_schistocytes_in_TTP_marked_in_blue_1.tif.jpg&w=640&q=50)
Upshaw–Schulman syndrome
Medical condition / From Wikipedia, the free encyclopedia
Dear Wikiwand AI, let's keep it short by simply answering these key questions:
Can you list the top facts and stats about Upshaw–Schulman syndrome?
Summarize this article for a 10 year old
Upshaw–Schulman syndrome (USS) is the recessively inherited form of thrombotic thrombocytopenic purpura (TTP), a rare and complex blood coagulation disease. USS is caused by the absence of the ADAMTS13 protease resulting in the persistence of ultra large von Willebrand factor multimers (ULVWF), causing episodes of acute thrombotic microangiopathy with disseminated multiple small vessel obstructions.[1][2] These obstructions deprive downstream tissues from blood and oxygen, which can result in tissue damage and death. The presentation of an acute USS episode is variable but usually associated with thrombocytopenia, microangiopathic hemolytic anemia (MAHA) with schistocytes on the peripheral blood smear,[3] fever and signs of ischemic organ damage in the brain, kidney and heart.
Upshaw–Schulman syndrome | |
---|---|
![]() | |
Blood smear under the microscope with typical schistocytes in TTP marked in blue – H&E stain | |
Specialty | Rheumatology ![]() |