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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 ]
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]
Immune thrombocytopenic purpura (ITP), also known as idiopathic thrombocytopenic purpura or immune thrombocytopenia, is an autoimmune primary disorder of hemostasis characterized by a low platelet count in the absence of other causes.
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]
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.
The classic TMAs are hemolytic uremic syndrome and thrombotic thrombocytopenic purpura. Other conditions with TMA include atypical hemolytic uremic syndrome , disseminated intravascular coagulation , scleroderma renal crisis, malignant hypertension , antiphospholipid antibody syndrome , and drug toxicities, e.g. calcineurin inhibitor toxicity.
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