For a patient with previously relapsed TTP but in clinical remission, what is the role of rituximab in treating asymptomatic ADAMTS13 deficiency to prevent relapse?

Rituximab has entered the treatment algorithm in the first-line setting for the initial treatment of TTP. However, in remission, the role of adjuvant rituximab or preemptive rituximab in response to declining ADAMTS13 levels is more nuanced. Should an asymptomatic patient, already treated with a 4-week course of rituximab, be retreated for a second occurrence of reduced ADATMS13 activity < 10%? When do you consider maintenance rituximab?

1 Answer

Mednet MemberInvited Expert

Hematology · Ohio State University

Answered on

A very good and interesting question. In remission, there is no question that severely deficient ADAMTS13 activity (<10%) is a strong risk factor for relapse. In patients with a chronic relapsing TTP history (at least 2 episodes), the approach most commonly has been to treat them preemptively with rituximab as has been reported in the literature by the French and English TTP research programs.

What is a bit trickier is the patient with only one episode of TTP previously and undetectable ADAMTS13 activity. Without the relapse history it has to be considered that there are patients who will not recover their ADAMTS13 activity but yet do not relapse, therefore preemptive treatment might not have been necessary. The problem is that the exact r...

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