Treatment

“[Plasma exchange] and immunosuppressors are pretty much ‘standard protocol’ for the treatment of acute TTP during pregnancy,” said co-author Barbara Ferrari, MD, of the Fondazione IRCCS Ca’ Granda-Ospedale Maggiore Policlinico, Angelo Bianchi Bonomi Hemophilia and Thrombosis Center in Milan, Italy. “On the contrary, I think there is some divergence for prophylaxis.”

If there is a decrease in ADAMTS13 during pregnancy, Dr Ferrari explained that the main difference would be the cut-off levels as to when the clinician decides to start any prophylaxis. “Moreover, not all medical centers can provide [plasma exchange] as prophylaxis in pregnancy,” she said.


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Aside from possible decisions on the delivery of the baby, treatment for pregnancy and nonpregnancy associated TTP are the same, although the former must take into account the effect of potential drug toxicity on the fetus. Therapeutic [plasma exchange] should be administered daily and initiated as soon as possible, but plasma infusion should only be considered if [plasma exchange] is not available. Among patients with autoimmune TTP, immunosuppressive therapy is also an integral part of the regimen with corticosteroids used as first line therapy. 

“I think there are not so many centers that use [plasma exchange] as prophylaxis in pregnancy but steroid are usually used in most of them,” said co-author Flora Peyvandi, MD, PhD, of the department of pathophysiology and transplantation at the University of Milan in Italy.

Overall, pregnancy associated TTP is a medical emergency for both the mother and baby, with specific disease and treatment related risks. A multidisciplinary approach is needed that involves hematologists and obstetricians, and a laboratory team with expertise in hemostasis, note the authors. “Moreover, women with a history of TTP who are planning pregnancy should be offered a counseling, as the risk of gravidic TTP recurrence needs to be considered.”

Disclosures: Some authors have declared affiliations with or received grant support from the pharmaceutical industry. Please refer to the original study for a full list of disclosures.

Reference

Ferrari B, Peyvandi F. How I treat thrombotic thrombocytopenic purpura in pregnancy. Blood. 2020;136(19):2125-2132. doi:1182.blood.2019000962