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Due to the high mortality of untreated TTP, a presumptive diagnosis of TTP is made even when only microangiopathic hemolytic anemia and thrombocytopenia are seen, and therapy is started. Transfusion is contraindicated in thrombotic TTP, as it fuels the coagulopathy. Since the early 1990s, plasmapheresis has become the treatment of choice for TTP.
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.
Thrombotic thrombocytopenic purpura (TTP) is a life-threatening disorder characterized by thrombocytopenia and microangiopathic hemolytic anemia accompanied by variable neurological dysfunction, kidney failure, and fever. It is caused by severely reduced activity of the von Willebrand factor-cleaving protease ADAMTS13.
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 ...
In February 2019, caplacizumab-yhdp (Cablivi, Ablynx NV) was approved in the United States for the treatment of adults with acquired thrombotic thrombocytopenic purpura (aTTP). The drug is used in combination with plasma exchange and immunosuppressive therapy.
Treatment of thrombotic thrombocytopenic purpura: Therapeutic plasma exchange with FFP/PF24 or thawed plasma as the replacement fluid is considered the treatment of choice for patients with proven or suspected thrombotic thrombocytopenic purpura (TTP).
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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