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Case Reports
. 2025 Jan 21;9(1):102687.
doi: 10.1016/j.rpth.2025.102687. eCollection 2025 Jan.

Prophylactic use of recombinant ADAMTS-13 during pregnancy for congenital thrombotic thrombocytopenic purpura

Éloïse Colliou  1 , Agnès Ribes  2 , Clotilde Gaible  1 , Mathilde Marlas  2 , David Ribes  1 , Isabelle Labadens  3 , Paul Guerby  4   5 , Stanislas Faguer  1   5   6
Affiliations
Case Reports

Prophylactic use of recombinant ADAMTS-13 during pregnancy for congenital thrombotic thrombocytopenic purpura

Éloïse Colliou et al. Res Pract Thromb Haemost. .

Abstract

Background: Congenital thrombotic thrombocytopenic purpura (cTTP) related to ADAMTS-13 deficiency is associated with a maternal risk of death of 10% and a risk of fetal loss greater than 50% without treatment.

Key clinical question: Is prophylactic use of recombinant (r)ADAMTS-13 during pregnancy in patients with cTTP safe and effective in preventing cTTP relapse?

Clinical approach: rADAMTS-13 was given intravenously weekly (40 Units/kg) from 17 weeks' gestation. ADAMTS-13 activity was undetectable before the first administration, reached 60% to 90% of normal levels 2 hours after, and became undetectable between days 4 and 6. A full dose was given in the hours preceding the delivery and on day 3. No flare-up of cTTP occurred during the pregnancy, and rADAMTS-13 was tolerated well. No anti-ADAMTS-13 antibodies developed.

Conclusion: Prophylactic use of rADAMTS-13 during pregnancy may prevent relapse of cTTP and reduce the risk of fetal loss, but an optimal regimen requires further attention.

Keywords: ADAMTS-13; congenital and thrombotic thrombocytopenic purpura; pregnancy; prophylaxis.

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Figures

Figure 1
Figure 1
(A) Kinetic profile of ADAMTS-13 activity (percentage of the normal value) after the first administration of TK755 (40 International Units [IU] per kilogram of weight; 3000 IU). (B) ADAMTS-13 activity during the period of ADAMTS-13 supplementation during pregnancy. ∗These values correspond to ADAMTS-13 activity measured 2 hours after the administration of TK755. The red arrow corresponds to the date of delivery. Green arrows correspond to infusions of recombinant ADAMTS-13 before and after delivery. rhADAMTS-13, recombinant human ADAMTS-13.
See this image and copyright information in PMC

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