How is thrombotic thrombocytopenic purpura (TTP) platelet disorder treated?

Updated: Aug 05, 2017
  • Author: Perumal Thiagarajan, MD; Chief Editor: Srikanth Nagalla, MBBS, MS, FACP  more...
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Answer

Answer

Thrombotic thrombocytopenic purpura (TTP) is a medical emergency, and prompt recognition and immediate initiation of plasma exchange is necessary. Until plasma exchange is instituted, fresh frozen plasma should be administered.

Plasma exchange therapy, introduced 30 years ago, has dramatically improved the prognosis for patients with TTP. Current mortality rates remain approximately 20%, compared with the greater than 90% mortality rate observed before the advent of plasma exchange therapy.

Plasma exchange (3-5 L/d) is instituted promptly and continued daily until the patient's platelet count is normalized and the LDH level is within the reference range. Several weeks of plasma exchange may be required before a durable remission is achieved.

Antiplatelet agents have not been shown to alter the natural history of the disease. Milder forms of TTP may respond to steroids.

Rituximab is now routinely recommended for treatment of acute TTP, typically in patients who have a suboptimal response to treatment, but also as first-line therapy. Early administration of rituximab is associated with faster remissions and fewer plasma exchanges. Rituximab therapy may also prevent relapses. [29]


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