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Thrombotic thrombocytopenic purpura (TTP) is a blood disorder that results in blood clots forming in small blood vessels throughout the body. [2] This results in a low platelet count , low red blood cells due to their breakdown , and often kidney , heart , and brain dysfunction. [ 1 ]
Treatment of thrombotic thrombocytopenic purpura (TTP) is a medical emergency, since the associated hemolytic anemia and platelet activation can lead to kidney failure and changes in the level of consciousness. Treatment of TTP was revolutionized in the 1980s with the application of plasmapheresis.
Apadamtase alfa, sold under the brand name Adzynma, is an enzyme replacement therapy used for the treatment of thrombotic thrombocytopenic purpura. [1] Apadamtase alfa is a human recombinant a disintegrin and metalloproteinase with thrombospondin motifs 13. [1] It is given by injection into a vein. [1]
By tradition, the term idiopathic thrombocytopenic purpura is used when the cause is idiopathic, or unknown. However, most cases are now considered to be immune-mediated. [citation needed] Another form is thrombotic thrombocytopenic purpura. [citation needed]
Thrombotic microangiopathy (TMA) is a pathology that results in thrombosis in capillaries and arterioles, due to an endothelial injury. [1] It may be seen in association with thrombocytopenia, anemia, purpura and kidney failure. The classic TMAs are hemolytic uremic syndrome and thrombotic thrombocytopenic purpura.
Cryosupernatant plasma can be used when replacement of FVIII is not required, [2] and is indicated for plasma exchange for patients with thrombotic thrombocytopenic purpura (TTP) [1] as well as for treatment of hemolytic-uremic syndrome (HUS) by plasma exchange, when plasma exchange is indicated. [3]
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