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Optimizing ADAMTS13 prophylaxis to reduce relapse and organ failure in congenital thrombotic thrombocytopenic purpura.

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Title: Optimizing ADAMTS13 prophylaxis to reduce relapse and organ failure in congenital thrombotic thrombocytopenic purpura.
Authors: Joly BS; Service d'Hématologie Biologique, Hôpital Lariboisière, Assistance Publique-Hôpitaux de Paris (AP-HP.Nord), Université Paris Cité, Paris, France.; Institut National de la Santé et de la Recherche Médicale, Unité Mixte de Recheche S1138, Centre de Recherche des Cordeliers, Université Paris Cité, Sorbonne Université, Paris, France., Joseph A; Service de Médecine Intensive Réanimation, Hôpital Ambroise Paré, Assistance Publique-Hôpitaux de Paris (AP-HP), Université de Versailles Saint-Quentin-en-Yvelines, Boulogne-Billancourt, France.; Institut National de la Santé et de la Recherche Médicale, Unité 1173, Infection et Inflammation, Université de Versailles Saint-Quentin-en-Yvelines, Université Paris Saclay, Guyancourt, France., Bouzid R; Institut National de la Santé et de la Recherche Médicale, Unité Mixte de Recheche S1138, Centre de Recherche des Cordeliers, Université Paris Cité, Sorbonne Université, Paris, France., Boisseau P; Service de Génétique Médicale, CHU de Nantes, Nantes, France., Kwon T; Service de Néphrologie Pédiatrique, Hôpital Robert-Debré, Assistance Publique-Hôpitaux de Paris (AP-HP.Nord), Université Paris Cité, Paris, France., Dossier C; Service de Néphrologie Pédiatrique, Hôpital Robert-Debré, Assistance Publique-Hôpitaux de Paris (AP-HP.Nord), Université Paris Cité, Paris, France., Gouge-Biebuyck N; Service de Néphrologie Pédiatrique, Hôpital Necker-Enfants Malades, Assistance Publique-Hôpitaux de Paris (AP-HP.Centre), Université Paris Cité, Paris, France., Boyer O; Service de Néphrologie Pédiatrique, Hôpital Necker-Enfants Malades, Assistance Publique-Hôpitaux de Paris (AP-HP.Centre), Université Paris Cité, Paris, France., Tsatsaris V; Service de Gynécologie-Obstétrique, Hôpital Cochin-Port Royal, Assistance Publique-Hôpitaux de Paris (AP-HP.Centre), Université Paris Cité, Paris, France., Harambat J; Service de Néphrologie Pédiatrique, Hôpital Pellegrin-Enfants, CHU de Bordeaux, Bordeaux, France., Delmas Y; Service de Néphrologie, Hôpital Pellegrin, Centre Hospitalo-Universitaire de Bordeaux, Bordeaux, France., Moranne O; Service de Néphrologie, Hôpital Carémeau, Centre Hospitalo-Universitaire de Nîmes, Institut Desbrest d'Épidémiologie et de Santé Publique, Université de Montpellier, Nîmes, France., Sellier-Leclerc AL; Service de Néphrologie Pédiatrique, Hôpital Femme Mère Enfants, Hospices Civils de Lyon, Bron, France., Marie M; Service de Médecine Interne, Hôpital Edouard Herriot, Hospices Civils de Lyon, Lyon, France., Barbet C; Service de Néphrologie, Hôpital Bretonneau, Centre Hospitalo-Universitaire de Tours, Tours, France., Provôt F; Service de Néphrologie, Hôpital Huriez, Centre Hospitalo-Universitaire de Lille, Lille, France., Benhamou Y; Service de Médecine Interne, Centre Hospitalo-Universitaire de Rouen, Rouen, France., Veyradier A; Service d'Hématologie Biologique, Hôpital Lariboisière, Assistance Publique-Hôpitaux de Paris (AP-HP.Nord), Université Paris Cité, Paris, France.; Institut National de la Santé et de la Recherche Médicale, Unité Mixte de Recheche S1138, Centre de Recherche des Cordeliers, Université Paris Cité, Sorbonne Université, Paris, France., Coppo P; Institut National de la Santé et de la Recherche Médicale, Unité Mixte de Recheche S1138, Centre de Recherche des Cordeliers, Université Paris Cité, Sorbonne Université, Paris, France.; Service d'Hématologie, Hôpital Saint-Antoine, Assistance Publique-Hôpitaux de Paris (AP-HP.6) Sorbonne Université, Paris, France., Augusto JF, Azoulay E, Barbay V, Benhamou Y, Arnaud J, Charvet-Rumpler A, Chauveau D, Choukroun G, Coindre JP, Coppo P, Delmas Y, Celia S, Dossier A, Simon V, Frémeaux-Bacchi V, Galicier L, Grangé S, Guidet B, Halimi JM, Antoine N, Jacobs F, Joly B, Kanouni T, Kaplanski G, Virginie R, Le Guern V, Moulin B, Rebibou JM, Ojeda Uribe M, Parquet N, Pène F, Perez P, Poullin P, Pouteil-Noble C, Presne C, Provôt F, Laurent M, Saheb S, Seguin A, Servais A, Stépanian A, Veyradier A, Vigneau C, Wynckel A, Zunic P, Krummel T, Ulrich M, Hertig A, Benoît S, Laurent G, Le Quintrec M, Kwon T, Chatelet V, Ducheyron D, Martis N, Marie M, Ribes D, Ronchetti AM
Source: Blood advances [Blood Adv] 2026 Mar 10; Vol. 10 (5), pp. 1831-1842.
Publication Type: Journal Article; Multicenter Study
Journal Info: Publisher: American Society of Hematology Country of Publication: United States NLM ID: 101698425 Publication Model: Print Cited Medium: Internet ISSN: 2473-9537 (Electronic) Linking ISSN: 24739529 NLM ISO Abbreviation: Blood Adv Subsets: MEDLINE
Database: MEDLINE Ultimate
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PubType: Academic Journal
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  Data: Optimizing ADAMTS13 prophylaxis to reduce relapse and organ failure in congenital thrombotic thrombocytopenic purpura.
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  Data: <searchLink fieldCode="AU" term="%22Joly+BS%22">Joly BS</searchLink>; Service d'Hématologie Biologique, Hôpital Lariboisière, Assistance Publique-Hôpitaux de Paris (AP-HP.Nord), Université Paris Cité, Paris, France.; Institut National de la Santé et de la Recherche Médicale, Unité Mixte de Recheche S1138, Centre de Recherche des Cordeliers, Université Paris Cité, Sorbonne Université, Paris, France.<br /><searchLink fieldCode="AU" term="%22Joseph+A%22">Joseph A</searchLink>; Service de Médecine Intensive Réanimation, Hôpital Ambroise Paré, Assistance Publique-Hôpitaux de Paris (AP-HP), Université de Versailles Saint-Quentin-en-Yvelines, Boulogne-Billancourt, France.; Institut National de la Santé et de la Recherche Médicale, Unité 1173, Infection et Inflammation, Université de Versailles Saint-Quentin-en-Yvelines, Université Paris Saclay, Guyancourt, France.<br /><searchLink fieldCode="AU" term="%22Bouzid+R%22">Bouzid R</searchLink>; Institut National de la Santé et de la Recherche Médicale, Unité Mixte de Recheche S1138, Centre de Recherche des Cordeliers, Université Paris Cité, Sorbonne Université, Paris, France.<br /><searchLink fieldCode="AU" term="%22Boisseau+P%22">Boisseau P</searchLink>; 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            NameFull: Presne C
      – PersonEntity:
          Name:
            NameFull: Provôt F
      – PersonEntity:
          Name:
            NameFull: Laurent M
      – PersonEntity:
          Name:
            NameFull: Saheb S
      – PersonEntity:
          Name:
            NameFull: Seguin A
      – PersonEntity:
          Name:
            NameFull: Servais A
      – PersonEntity:
          Name:
            NameFull: Stépanian A
      – PersonEntity:
          Name:
            NameFull: Veyradier A
      – PersonEntity:
          Name:
            NameFull: Vigneau C
      – PersonEntity:
          Name:
            NameFull: Wynckel A
      – PersonEntity:
          Name:
            NameFull: Zunic P
      – PersonEntity:
          Name:
            NameFull: Krummel T
      – PersonEntity:
          Name:
            NameFull: Ulrich M
      – PersonEntity:
          Name:
            NameFull: Hertig A
      – PersonEntity:
          Name:
            NameFull: Benoît S
      – PersonEntity:
          Name:
            NameFull: Laurent G
      – PersonEntity:
          Name:
            NameFull: Le Quintrec M
      – PersonEntity:
          Name:
            NameFull: Kwon T
      – PersonEntity:
          Name:
            NameFull: Chatelet V
      – PersonEntity:
          Name:
            NameFull: Ducheyron D
      – PersonEntity:
          Name:
            NameFull: Martis N
      – PersonEntity:
          Name:
            NameFull: Marie M
      – PersonEntity:
          Name:
            NameFull: Ribes D
      – PersonEntity:
          Name:
            NameFull: Ronchetti AM
    IsPartOfRelationships:
      – BibEntity:
          Dates:
            – D: 10
              M: 03
              Text: 2026 Mar 10
              Type: published
              Y: 2026
          Identifiers:
            – Type: issn-electronic
              Value: 2473-9537
          Numbering:
            – Type: volume
              Value: 10
            – Type: issue
              Value: 5
          Titles:
            – TitleFull: Blood advances
              Type: main
ResultId 1