From the Research
Recombinant ADAMTS-13 can be used during pregnancy for women with congenital or acquired thrombotic thrombocytopenic purpura (TTP), with plasma exchange as first-line therapy and rADAMTS-13 considered in cases where plasma exchange is ineffective or unavailable. The use of rADAMTS-13 in pregnancy is supported by recent studies, including a 2024 study published in the Journal of Thrombosis and Haemostasis, which discusses the potential benefits of targeted ADAMTS-13 replacement therapies for TTP 1. When used, rADAMTS-13 is typically administered intravenously at doses ranging from 10-40 units/kg, with frequency determined by clinical response and ADAMTS-13 activity levels.
During pregnancy, more frequent monitoring of ADAMTS-13 levels is recommended (every 1-2 weeks), with dose adjustments made accordingly. Treatment should be initiated promptly when ADAMTS-13 activity falls below 10% or with clinical signs of TTP. The safety profile of rADAMTS-13 appears favorable for use during pregnancy as it replaces the naturally occurring enzyme without crossing the placenta in significant amounts. This treatment works by replacing the deficient ADAMTS-13 enzyme, which is responsible for cleaving ultra-large von Willebrand factor multimers, thereby preventing the formation of platelet-rich microthrombi that characterize TTP and can lead to serious maternal and fetal complications if left untreated.
Some studies have discussed the pathophysiology and management of TTP, including a 2021 study published in the Journal of Clinical Medicine, which reviews the current understanding of TTP and its treatment options 2. Another study published in 2018 in the Journal of Hematology discusses the complex association between HELLP syndrome and TTP, and proposes a stepwise practical plan for differential diagnosis 3. However, the most recent and highest-quality study on this topic is the 2024 study published in the Journal of Thrombosis and Haemostasis, which provides the most up-to-date information on the use of rADAMTS-13 in pregnancy 1.
Key points to consider when using rADAMTS-13 in pregnancy include:
- Monitoring ADAMTS-13 levels every 1-2 weeks
- Adjusting doses based on clinical response and ADAMTS-13 activity levels
- Initiating treatment promptly when ADAMTS-13 activity falls below 10% or with clinical signs of TTP
- Using plasma exchange as first-line therapy, with rADAMTS-13 considered in cases where plasma exchange is ineffective or unavailable.