What is the treatment for thrombotic thrombocytopenic purpura?

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What is the treatment for thrombotic thrombocytopenic purpura?

In thrombotic thrombocytopenic purpura there is a severe deficiency of the enzyme ADAMTS13, leading to accumulation of ultra-large von Willebrand factor multimers and widespread microthrombi that consume platelets. The most effective treatment is urgent plasma exchange with plasma replacement, which simultaneously removes circulating autoantibodies against ADAMTS13 and replenishes functional ADAMTS13. This rapidly halts ongoing platelet consumption and microthrombosis, improving survival when started promptly. Steroids may be added to help suppress antibody production, but they do not by themselves reverse the process. Platelet transfusions are generally avoided because they can fuel the formation of more microthrombi, except in cases of life-threatening bleeding where bleeding control is the priority. Antibiotics do not treat the underlying disorder.

In thrombotic thrombocytopenic purpura there is a severe deficiency of the enzyme ADAMTS13, leading to accumulation of ultra-large von Willebrand factor multimers and widespread microthrombi that consume platelets. The most effective treatment is urgent plasma exchange with plasma replacement, which simultaneously removes circulating autoantibodies against ADAMTS13 and replenishes functional ADAMTS13. This rapidly halts ongoing platelet consumption and microthrombosis, improving survival when started promptly.

Steroids may be added to help suppress antibody production, but they do not by themselves reverse the process. Platelet transfusions are generally avoided because they can fuel the formation of more microthrombi, except in cases of life-threatening bleeding where bleeding control is the priority. Antibiotics do not treat the underlying disorder.

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