For Publishers
DiscoveryMetadataPeer reviewHostingPublishing
For Researchers
JoinPublishReviewCollect
Register
Blog
About
Search
Advanced search
My ScienceOpen
Search
For Publishers
For Researchers
Blog
About
6
views
22
references
 Top references
cited by
0
    
0 reviews
 Review
0
comments
 Comment
0
recommends
+1 Recommend
0 collections
    0
    shares
      718
      similar
       All similar
      • Record: found
      • Abstract: found
      • Article: found
      Is Open Access

      Recombinant ADAMTS13 for Immune Thrombotic Thrombocytopenic Purpura

      research-article
      Author(s): , M.D. 1 , 2 , 3 , 4 , 5 , , D.Phil. 6 , 7 , , M.D., Ph.D. 8 , , M.D., Ph.D. 5 , 6 , , B.A. 3 , , B.S. 3 , , B.A. 8 , , M.D., Ph.D. 5 , 6 , , M.D., M.P.H. 5 , 9 , , M.D., M.B.A. 5 , 9 , , M.D. 3 , 5 , , B.S., B.A. 3 , 5 , , M.D. 5 , 10 , , M.D., Ph.D. 5 , 6 , , M.D. 11 , , M.D. 12 , , M.D. 13 , , Ph.D., M.D. 13 , , D.O., M.P.H. 1 , 5 , , M.D. 8 , , M.D., D.Phil. 1 , 5 , , M.D. 2 , 5 , , M.D. 5 , 6 , , M.D. 1 , 2 , 5 , , M.D., Ph.D. 1 , 5 , , M.D., Ph.D. 1 , 5
      Publication date PMC-release:
      Journal: The New England journal of medicine

      Read this article at

      ScienceOpenPublisherPMC
      Bookmark
          There is no author summary for this article yet. Authors can add summaries to their articles on ScienceOpen to make them more accessible to a non-specialist audience.

          Summary

          In immune thrombotic thrombocytopenic purpura (iTTP), autoantibodies against the metalloprotease ADAMTS13 lead to catastrophic microvascular thrombosis. However, the potential benefits of recombinant human ADAMTS13 (rADAMTS13) in iTTP remain unknown. We report the clinical use of rADAMTS13 in iTTP. Administration of rADAMTS13 was associated with rapid suppression of disease activity and complete recovery in a critically ill patient whose condition had proven refractory to all available treatments. We show that rADAMTS13 causes immune complex formation, saturating the autoantibody and likely promoting its clearance. Our data support the role of rADAMTS13 as a novel adjunctive therapy in iTTP.

          Related collections

          Most cited references22

          • Record: found
          • Abstract: found
          • Article: not found

          Syndromes of thrombotic microangiopathy.

          James N. George, Carla Nester (2014)
          This review article covers the diverse pathophysiological pathways that can lead to microangiopathic hemolytic anemia and a procoagulant state with or without damage to the kidneys and other organs.
          Article 0 comments Cited 351 times – based on 0 reviews     Review now
            Bookmark
            • Record: found
            • Abstract: found
            • Article: not found

            Derivation and external validation of the PLASMIC score for rapid assessment of adults with thrombotic microangiopathies: a cohort study.

            Pavan K Bendapudi, Shelley Hurwitz, Ashley Fry … (2017)
            Among the syndromes characterised by thrombotic microangiopathy, thrombotic thrombocytopenic purpura is distinguished by a severe deficiency in the ADAMTS13 enzyme. Patients with this disorder need urgent treatment with plasma exchange. Because ADAMTS13 activity testing typically requires prolonged turnaround times and might be unavailable in resource-poor settings, a method to rapidly assess the likelihood of severe ADAMTS13 deficiency is needed.
            Article 0 comments Cited 119 times – based on 0 reviews     Review now
              Bookmark
              • Record: found
              • Abstract: not found
              • Article: not found

              ISTH guidelines for treatment of thrombotic thrombocytopenic purpura

              X Long Zheng, Sara K. Vesely, Spero R. Cataland … (2020)
              Article 0 comments Cited 100 times – based on 0 reviews     Review now
                Bookmark
                All references

                Author and article information

                Journal
                Journal ID (nlm-journal-id): 0255562
                Journal ID (pubmed-jr-id): 5985
                Journal ID (nlm-ta): N Engl J Med
                Journal ID (iso-abbrev): N Engl J Med
                Title: The New England journal of medicine
                ISSN (Print): 0028-4793
                ISSN (Electronic): 1533-4406
                Publication date Nihms-submitted: 22 May 2024
                Publication date (Print): 09 May 2024
                Publication date PMC-release: 26 May 2024
                Volume: 390
                Issue: 18
                Pages: 1690-1698
                Affiliations
                [1. ]Blood Transfusion Service, Massachusetts General Hospital, Boston, MA
                [2. ] Division of Hematology, Massachusetts General Hospital, Boston, MA
                [3. ]Division of Hemostasis and Thrombosis, Beth Israel Deaconess Medical Center, Boston, MA
                [4. ]The Broad Institute of MIT and Harvard, Cambridge, MA
                [5. ]Harvard Medical School, Boston, MA
                [6. ]Department of Pathology, Massachusetts General Hospital, Boston, MA
                [7. ]Department of Laboratory Medicine & Pathology, University of Washington, Seattle, WA
                [8. ]Versiti Blood Center of Wisconsin, Milwaukee, WI
                [9. ]Cardiology Division, Massachusetts General Hospital, Boston, MA
                [10. ]Division of Hematology and Apheresis Service, Beth Israel Deaconess Medical Center, Boston, MA
                [11. ]Division of Hematology and Oncology, Lahey Hospital and Medical Center, Burlington, MA
                [12. ]Department of Hematology and Clinical Oncology, UMass Chan Medical School-Baystate Health, Springfield, MA
                [13. ]Department of Pathology, Transfusion/Apheresis Medicine Services, UMass Chan Medical School-Baystate Health, Springfield, MA
                Author notes
                Corresponding Author: Pavan K. Bendapudi, M.D., Center for Life Science, Room 906, 3 Blackfan Circle, Boston, MA 02115, pbendapudi@ 123456mgb.org
                Article
                Manuscript ID: NIHMS1983589
                DOI: 10.1056/NEJMoa2402567
                PMC ID: 11128310
                PubMed ID: 38718359
                SO-VID: 62e197e8-cd77-4482-9bf0-fcf5491b1393
                License:

                This Author Accepted Manuscript is licensed for use under the CC-BY-NC-ND license.

                History
                Categories
                Subject: Article

                Data availability:

                Comments

                Comment on this article

                scite_

                Similar content718

                See all similar

                Most referenced authors612

                See all reference authors