Prophylactic use of recombinant ADAMTS-13 during pregnancy for congenital thrombotic thrombocytopenic purpura
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...
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Elsevier
2025-01-01
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| Series: | Research and Practice in Thrombosis and Haemostasis |
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| Online Access: | http://www.sciencedirect.com/science/article/pii/S2475037925000111 |
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| author | Éloïse Colliou Agnès Ribes Clotilde Gaible Mathilde Marlas David Ribes Isabelle Labadens Paul Guerby Stanislas Faguer |
| author_facet | Éloïse Colliou Agnès Ribes Clotilde Gaible Mathilde Marlas David Ribes Isabelle Labadens Paul Guerby Stanislas Faguer |
| author_sort | Éloïse Colliou |
| collection | DOAJ |
| description | 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. |
| format | Article |
| id | doaj-art-78674dade80441f194724764e6c7353b |
| institution | Kabale University |
| issn | 2475-0379 |
| language | English |
| publishDate | 2025-01-01 |
| publisher | Elsevier |
| record_format | Article |
| series | Research and Practice in Thrombosis and Haemostasis |
| spelling | doaj-art-78674dade80441f194724764e6c7353b2025-02-08T05:00:47ZengElsevierResearch and Practice in Thrombosis and Haemostasis2475-03792025-01-0191102687Prophylactic use of recombinant ADAMTS-13 during pregnancy for congenital thrombotic thrombocytopenic purpuraÉloïse Colliou0Agnès Ribes1Clotilde Gaible2Mathilde Marlas3David Ribes4Isabelle Labadens5Paul Guerby6Stanislas Faguer7Department of Nephrology and Organ Transplantation, National Referral Centre for Rare Kidney Diseases, University Hospital of Toulouse, Toulouse, FranceLaboratory for Hematology and Hemostasis, University Hospital of Toulouse, Toulouse, FranceDepartment of Nephrology and Organ Transplantation, National Referral Centre for Rare Kidney Diseases, University Hospital of Toulouse, Toulouse, FranceLaboratory for Hematology and Hemostasis, University Hospital of Toulouse, Toulouse, FranceDepartment of Nephrology and Organ Transplantation, National Referral Centre for Rare Kidney Diseases, University Hospital of Toulouse, Toulouse, FranceDepartment of Clinical Pharmacology, University Hospital of Toulouse, Toulouse, FranceDepartment of Obstetrics, University Hospital of Toulouse, Toulouse, France; Faculty of Health, University Toulouse-3, Toulouse, FranceDepartment of Nephrology and Organ Transplantation, National Referral Centre for Rare Kidney Diseases, University Hospital of Toulouse, Toulouse, France; Faculty of Health, University Toulouse-3, Toulouse, France; Institute for Metabolic and Cardiovascular Diseases (Unit 1297), National Institute for Health and Medical Research, Toulouse, France; Correspondence Stanislas Faguer, Department of Nephrology and Organ Transplantation, University Hospital of Toulouse, 1 Avenue du Pr. Jean Poulhes, 31059 Toulouse Cedex, France.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.http://www.sciencedirect.com/science/article/pii/S2475037925000111ADAMTS-13congenital and thrombotic thrombocytopenic purpurapregnancyprophylaxis |
| spellingShingle | Éloïse Colliou Agnès Ribes Clotilde Gaible Mathilde Marlas David Ribes Isabelle Labadens Paul Guerby Stanislas Faguer Prophylactic use of recombinant ADAMTS-13 during pregnancy for congenital thrombotic thrombocytopenic purpura Research and Practice in Thrombosis and Haemostasis ADAMTS-13 congenital and thrombotic thrombocytopenic purpura pregnancy prophylaxis |
| title | Prophylactic use of recombinant ADAMTS-13 during pregnancy for congenital thrombotic thrombocytopenic purpura |
| title_full | Prophylactic use of recombinant ADAMTS-13 during pregnancy for congenital thrombotic thrombocytopenic purpura |
| title_fullStr | Prophylactic use of recombinant ADAMTS-13 during pregnancy for congenital thrombotic thrombocytopenic purpura |
| title_full_unstemmed | Prophylactic use of recombinant ADAMTS-13 during pregnancy for congenital thrombotic thrombocytopenic purpura |
| title_short | Prophylactic use of recombinant ADAMTS-13 during pregnancy for congenital thrombotic thrombocytopenic purpura |
| title_sort | prophylactic use of recombinant adamts 13 during pregnancy for congenital thrombotic thrombocytopenic purpura |
| topic | ADAMTS-13 congenital and thrombotic thrombocytopenic purpura pregnancy prophylaxis |
| url | http://www.sciencedirect.com/science/article/pii/S2475037925000111 |
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