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von Willebrand factor neutralizing and non-neutralizing alloantibodies in 213 subjects with type 3 von Willebrand disease enrolled in 3WINTERS-IPS.
Pagliari, Maria Teresa; Budde, Ulrich; Baronciani, Luciano; Eshghi, Peyman; Ahmadinejad, Minoo; Badiee, Zahra; Baghaipour, Mohammad-Reza; Benítez Hidalgo, Olga; Biguzzi, Eugenia; Bodó, Imre; Castaman, Giancarlo; Goudemand, Jenny; Karimi, Mehran; Keikhaei, Bijan; Lassila, Riitta; Leebeek, Frank W G; Lopez Fernandez, Maria Fernanda; Marino, Renato; Oldenburg, Johannes; Peake, Ian; Santoro, Cristina; Schneppenheim, Reinhard; Tiede, Andreas; Toogeh, Gholamreza; Tosetto, Alberto; Trossaert, Marc; Yadegari, Hamideh; Zetterberg, Eva M K; Mannucci, Pier Mannuccio; Federici, Augusto B; Eikenboom, Jeroen; Peyvandi, Flora.
Afiliación
  • Pagliari MT; Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Angelo Bianchi Bonomi Hemophilia and Thrombosis Center, Milan, Italy.
  • Budde U; Hemostaseology Medilys Laborgesellschaft mbH, Hamburg, Germany.
  • Baronciani L; Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Angelo Bianchi Bonomi Hemophilia and Thrombosis Center, Milan, Italy.
  • Eshghi P; Pediatric Congenital Hematologic Disorders Research Center, Shahid Beheshti University of Medical Sciences, Tehran, Iran.
  • Ahmadinejad M; Pediatric Congenital Hematologic Disorders Research Center, Shahid Beheshti University of Medical Sciences, Tehran, Iran; Blood Transfusion Research Center, High Institute for Research and Education in Transfusion Medicine, Tehran, Iran.
  • Badiee Z; Hemophilia-Thalassemia Center, Mashhad University of Medical Science, Mashad, Iran.
  • Baghaipour MR; Iranian Hemophilia Comprehensive Treatment Centre, Tehran, Islamic Republic of Iran.
  • Benítez Hidalgo O; Hemophilia Unit, Hematology Department, Hospital Universitari Vall d'Hebron, Spain.
  • Biguzzi E; Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Angelo Bianchi Bonomi Hemophilia and Thrombosis Center, Milan, Italy.
  • Bodó I; Department of Internal Medicine and Hematology - Semmelweis University, Budapest, Hungary.
  • Castaman G; Center for Bleeding Disorders and Coagulation, Careggi University Hospital, Florence, Italy.
  • Goudemand J; Department of Hematology and Transfusion, University of Lille, CHU Lille, Lille, France.
  • Karimi M; Hematology Research Center, Nemazee Hospital, Shiraz University of Medical Science, Shiraz, Iran.
  • Keikhaei B; Thalassemia and Hemoglobinopathy Research Center, Health Research Institute, Ahvaz Jundishapur, University of Medical Sciences, Ahvaz, Iran.
  • Lassila R; Research Program Unit in Oncology, University of Helsinki, Helsinki University Central Hospital, Coagulation disorders, Helsinki, Finland.
  • Leebeek FWG; Department of Hematology, Erasmus MC, University Medical Center Rotterdam, Rotterdam, the Netherlands.
  • Lopez Fernandez MF; Complejo Hospitalario Universitario de A Coruña - Servicio de Hematología y Hemoterapia, A Coruña, Spain.
  • Marino R; Hemophilia and Thrombosis Centre, University Hospital Policlinico, Bari, Italy.
  • Oldenburg J; Institute of Experimental Haematology and Transfusion Medicine, University Hospital Bonn, Bonn, Germany.
  • Peake I; Faculty of Medicine, Dentistry and Health, University of Sheffield, Sheffield, United Kingdom.
  • Santoro C; Hematology, Hemophilia and Thrombosis Center, University Hospital Policlinico Umberto I, Rome, Italy.
  • Schneppenheim R; Department of Pediatric Hematology and Oncology, University Medical Centre, Hamburg-Eppendorf, Hamburg, Germany.
  • Tiede A; Department of Hematology, Hemostasis, Oncology and Stem Cell Transplantation, Hannover Medical School, Hannover, Germany.
  • Toogeh G; Thrombosis Hemostasis Research Center, Tehran University of Medical Sciences, Tehran, Iran.
  • Tosetto A; Hemophilia and Thrombosis Center, Hematology Department, San Bortolo Hospital, Vicenza, Italy.
  • Trossaert M; Centre Régional de Traitement de l'Hémophilie - Laboratoire d'Hématologie, Nantes, France.
  • Yadegari H; Institute of Experimental Haematology and Transfusion Medicine, University Hospital Bonn, Bonn, Germany.
  • Zetterberg EMK; Skane University Hospital, Malmo, Sweden.
  • Mannucci PM; Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Angelo Bianchi Bonomi Hemophilia and Thrombosis Center, Milan, Italy.
  • Federici AB; Department of Oncology and Oncohematology,Hematology and Transfusion Medicine, L. Sacco University Hospital, University of Milan, Milan, Italy.
  • Eikenboom J; Department of Internal Medicine, Division of Thrombosis and Hemostasis, Leiden University Medical Center, Leiden, the Netherlands.
  • Peyvandi F; Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Angelo Bianchi Bonomi Hemophilia and Thrombosis Center, Milan, Italy; Department of Pathophysiology and Transplantation, Università degli Studi di Milano, Milan, Italy. Electronic address: flora.peyvandi@unimi.it.
J Thromb Haemost ; 21(4): 787-799, 2023 04.
Article en En | MEDLINE | ID: mdl-36792472
BACKGROUND: Type 3 von Willebrand disease (VWD) is the most severe form of this disease owing to the almost complete deficiency of von Willebrand factor (VWF). Replacement therapy with plasma-derived products containing VWF or recombinant VWF rarely cause the development of alloantibodies against VWF that may be accompanied by anaphylactic reactions. OBJECTIVE: The objective of this study was to assess the prevalence of anti-VWF alloantibodies in subjects with type 3 VWD enrolled in the 3WINTERS-IPS. METHODS: An indirect in-house enzyme-linked immunosorbent assay has been used to test all the alloantibodies against VWF. Neutralizing antibodies (inhibitors) have been tested with a Bethesda-based method by using a VWF collagen binding (VWF:CB) assay. Samples positive for anti-VWF antibodies were further tested with Bethesda-based methods by using the semiautomated gain-of-function glycoprotein-Ib binding (VWF:GPIbM) and a VWF antigen (VWF:Ag) enzyme-linked immunosorbent assay. RESULTS: In total, 18 of the 213 (8.4%) subjects tested positive for anti-VWF antibodies and 13 of 213 (6%) had VWF:CB inhibitors. These 13 were among the 18 with anti-VWF antibodies. Of the 5 without VWF:CB inhibitors, 3 had non-neutralizing antibodies, 1 only inhibitor against VWF:GPIbM, and one could not be tested further. Ten of the 13 subjects with VWF:CB inhibitors also had VWF:GPIbM inhibitors, 6 of whom also had VWF:Ag inhibitors. Subjects with inhibitors were homozygous for VWF null alleles (11/14), homozygous for a missense variant (1/14), or partially characterized (2/14). CONCLUSIONS: Anti-VWF antibodies were found in 8.4% of subjects with type 3 VWD, whereas neutralizing VWF inhibitors were found in 6%, mainly in subjects homozygous for VWF null alleles. Because inhibitors may be directed toward different VWF epitopes, their detection is dependent on the assay used.
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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Enfermedades de von Willebrand / Enfermedad de von Willebrand Tipo 2 / Enfermedad de von Willebrand Tipo 3 Tipo de estudio: Diagnostic_studies / Risk_factors_studies Límite: Humans Idioma: En Revista: J Thromb Haemost Asunto de la revista: HEMATOLOGIA Año: 2023 Tipo del documento: Article País de afiliación: Italia Pais de publicación: Reino Unido
Texto completo
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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Enfermedades de von Willebrand / Enfermedad de von Willebrand Tipo 2 / Enfermedad de von Willebrand Tipo 3 Tipo de estudio: Diagnostic_studies / Risk_factors_studies Límite: Humans Idioma: En Revista: J Thromb Haemost Asunto de la revista: HEMATOLOGIA Año: 2023 Tipo del documento: Article País de afiliación: Italia Pais de publicación: Reino Unido