von Willebrand disease: laboratory aspects of diagnosis and treatment
Identifieur interne : 000F76 ( Istex/Corpus ); précédent : 000F75; suivant : 000F77von Willebrand disease: laboratory aspects of diagnosis and treatment
Auteurs : E. J. Favaloro ; D. Lillicrap ; M. A. Lazzari ; M. Cattaneo ; C. Mazurier ; A. Woods ; S. Meschengieser ; A. Blanco ; A. C. Kempfer ; A. Hubbard ; A. ChangSource :
- Haemophilia [ 1351-8216 ] ; 2004-10.
English descriptors
- KwdEn :
- Assay, Blackwell publishing, Blood group, Caesarean section, Clinical pathology, Collagen binding, Excessive mucocutaneous, Family history, Favaloro, Fviii, Fviii level, Fviii levels, Genetic analysis, Haemophilia, Inadequate response, Laboratoire francais, Laboratory aspects, Medical research, Menstrual cycle, Platelet, Possible type, Quantitative defect, Severe type, Thromb haemost, Willebrand, Willebrand disease, Willebrand factor.
- Teeft :
- Assay, Blackwell publishing, Blood group, Caesarean section, Clinical pathology, Collagen binding, Excessive mucocutaneous, Family history, Favaloro, Fviii, Fviii level, Fviii levels, Genetic analysis, Haemophilia, Inadequate response, Laboratoire francais, Laboratory aspects, Medical research, Menstrual cycle, Platelet, Possible type, Quantitative defect, Severe type, Thromb haemost, Willebrand, Willebrand disease, Willebrand factor.
Abstract
Summary. von Willebrand disease is the most common inherited bleeding disorder in humans. VWD can be classified into three major types, designated Types 1, 2 and 3; Type 2 can be further separated into subtypes 2A, 2B, 2M and 2N. The diagnosis of VWD requires a personal and family history of bleeding and confirmation by laboratory analysis. Although Types 2 and 3 are relatively straightforward to diagnose, there may be a risk of overdiagnosis of Type 1 because of an overlap within the normal range. We also report on the clinical profile and diagnosis of VWD in a South American cohort of patients and on the in vitro characteristics of some factor concentrates available for treatment of VWD.
Url:
DOI: 10.1111/j.1365-2516.2004.00979.x
Links to Exploration step
ISTEX:5380B00AA14E1F9E62415DC83D19AA5A8F69EBA5Le document en format XML
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<affiliation><mods:affiliation>*Institute of Clinical Pathology and Medical Research, Australia; †Department of Pathology and Molecular Medicine, Richardson Laboratory, Queen's University, Canada; ‡Instituto de Investigaciones Hematológicas, Academia Nacional de Medicina, Argentina; §University of Milano, Ospedale San Paolo, Unit di Hematologica, Italy; ¶Department of Preclinical Development, Laboratoire Français du Fractionnement et des Biotechnologies, France; ††National Institute for Biological Standards and Control, UK, and ‡‡Center for Biologics Evaluation and Research, Food and Drug Administration, USA</mods:affiliation>
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<affiliation><mods:affiliation>*Institute of Clinical Pathology and Medical Research, Australia; †Department of Pathology and Molecular Medicine, Richardson Laboratory, Queen's University, Canada; ‡Instituto de Investigaciones Hematológicas, Academia Nacional de Medicina, Argentina; §University of Milano, Ospedale San Paolo, Unit di Hematologica, Italy; ¶Department of Preclinical Development, Laboratoire Français du Fractionnement et des Biotechnologies, France; ††National Institute for Biological Standards and Control, UK, and ‡‡Center for Biologics Evaluation and Research, Food and Drug Administration, USA</mods:affiliation>
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<affiliation><mods:affiliation>*Institute of Clinical Pathology and Medical Research, Australia; †Department of Pathology and Molecular Medicine, Richardson Laboratory, Queen's University, Canada; ‡Instituto de Investigaciones Hematológicas, Academia Nacional de Medicina, Argentina; §University of Milano, Ospedale San Paolo, Unit di Hematologica, Italy; ¶Department of Preclinical Development, Laboratoire Français du Fractionnement et des Biotechnologies, France; ††National Institute for Biological Standards and Control, UK, and ‡‡Center for Biologics Evaluation and Research, Food and Drug Administration, USA</mods:affiliation>
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<author><name sortKey="Woods, A" sort="Woods, A" uniqKey="Woods A" first="A." last="Woods">A. Woods</name>
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<author><name sortKey="Meschengieser, S" sort="Meschengieser, S" uniqKey="Meschengieser S" first="S." last="Meschengieser">S. Meschengieser</name>
<affiliation><mods:affiliation>*Institute of Clinical Pathology and Medical Research, Australia; †Department of Pathology and Molecular Medicine, Richardson Laboratory, Queen's University, Canada; ‡Instituto de Investigaciones Hematológicas, Academia Nacional de Medicina, Argentina; §University of Milano, Ospedale San Paolo, Unit di Hematologica, Italy; ¶Department of Preclinical Development, Laboratoire Français du Fractionnement et des Biotechnologies, France; ††National Institute for Biological Standards and Control, UK, and ‡‡Center for Biologics Evaluation and Research, Food and Drug Administration, USA</mods:affiliation>
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<author><name sortKey="Blanco, A" sort="Blanco, A" uniqKey="Blanco A" first="A." last="Blanco">A. Blanco</name>
<affiliation><mods:affiliation>*Institute of Clinical Pathology and Medical Research, Australia; †Department of Pathology and Molecular Medicine, Richardson Laboratory, Queen's University, Canada; ‡Instituto de Investigaciones Hematológicas, Academia Nacional de Medicina, Argentina; §University of Milano, Ospedale San Paolo, Unit di Hematologica, Italy; ¶Department of Preclinical Development, Laboratoire Français du Fractionnement et des Biotechnologies, France; ††National Institute for Biological Standards and Control, UK, and ‡‡Center for Biologics Evaluation and Research, Food and Drug Administration, USA</mods:affiliation>
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<author><name sortKey="Kempfer, A C" sort="Kempfer, A C" uniqKey="Kempfer A" first="A. C." last="Kempfer">A. C. Kempfer</name>
<affiliation><mods:affiliation>*Institute of Clinical Pathology and Medical Research, Australia; †Department of Pathology and Molecular Medicine, Richardson Laboratory, Queen's University, Canada; ‡Instituto de Investigaciones Hematológicas, Academia Nacional de Medicina, Argentina; §University of Milano, Ospedale San Paolo, Unit di Hematologica, Italy; ¶Department of Preclinical Development, Laboratoire Français du Fractionnement et des Biotechnologies, France; ††National Institute for Biological Standards and Control, UK, and ‡‡Center for Biologics Evaluation and Research, Food and Drug Administration, USA</mods:affiliation>
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<author><name sortKey="Hubbard, A" sort="Hubbard, A" uniqKey="Hubbard A" first="A." last="Hubbard">A. Hubbard</name>
<affiliation><mods:affiliation>*Institute of Clinical Pathology and Medical Research, Australia; †Department of Pathology and Molecular Medicine, Richardson Laboratory, Queen's University, Canada; ‡Instituto de Investigaciones Hematológicas, Academia Nacional de Medicina, Argentina; §University of Milano, Ospedale San Paolo, Unit di Hematologica, Italy; ¶Department of Preclinical Development, Laboratoire Français du Fractionnement et des Biotechnologies, France; ††National Institute for Biological Standards and Control, UK, and ‡‡Center for Biologics Evaluation and Research, Food and Drug Administration, USA</mods:affiliation>
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<author><name sortKey="Chang, A" sort="Chang, A" uniqKey="Chang A" first="A." last="Chang">A. Chang</name>
<affiliation><mods:affiliation>*Institute of Clinical Pathology and Medical Research, Australia; †Department of Pathology and Molecular Medicine, Richardson Laboratory, Queen's University, Canada; ‡Instituto de Investigaciones Hematológicas, Academia Nacional de Medicina, Argentina; §University of Milano, Ospedale San Paolo, Unit di Hematologica, Italy; ¶Department of Preclinical Development, Laboratoire Français du Fractionnement et des Biotechnologies, France; ††National Institute for Biological Standards and Control, UK, and ‡‡Center for Biologics Evaluation and Research, Food and Drug Administration, USA</mods:affiliation>
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<author><name sortKey="Favaloro, E J" sort="Favaloro, E J" uniqKey="Favaloro E" first="E. J." last="Favaloro">E. J. Favaloro</name>
<affiliation><mods:affiliation>*Institute of Clinical Pathology and Medical Research, Australia; †Department of Pathology and Molecular Medicine, Richardson Laboratory, Queen's University, Canada; ‡Instituto de Investigaciones Hematológicas, Academia Nacional de Medicina, Argentina; §University of Milano, Ospedale San Paolo, Unit di Hematologica, Italy; ¶Department of Preclinical Development, Laboratoire Français du Fractionnement et des Biotechnologies, France; ††National Institute for Biological Standards and Control, UK, and ‡‡Center for Biologics Evaluation and Research, Food and Drug Administration, USA</mods:affiliation>
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<author><name sortKey="Lillicrap, D" sort="Lillicrap, D" uniqKey="Lillicrap D" first="D." last="Lillicrap">D. Lillicrap</name>
<affiliation><mods:affiliation>*Institute of Clinical Pathology and Medical Research, Australia; †Department of Pathology and Molecular Medicine, Richardson Laboratory, Queen's University, Canada; ‡Instituto de Investigaciones Hematológicas, Academia Nacional de Medicina, Argentina; §University of Milano, Ospedale San Paolo, Unit di Hematologica, Italy; ¶Department of Preclinical Development, Laboratoire Français du Fractionnement et des Biotechnologies, France; ††National Institute for Biological Standards and Control, UK, and ‡‡Center for Biologics Evaluation and Research, Food and Drug Administration, USA</mods:affiliation>
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<author><name sortKey="Lazzari, M A" sort="Lazzari, M A" uniqKey="Lazzari M" first="M. A." last="Lazzari">M. A. Lazzari</name>
<affiliation><mods:affiliation>*Institute of Clinical Pathology and Medical Research, Australia; †Department of Pathology and Molecular Medicine, Richardson Laboratory, Queen's University, Canada; ‡Instituto de Investigaciones Hematológicas, Academia Nacional de Medicina, Argentina; §University of Milano, Ospedale San Paolo, Unit di Hematologica, Italy; ¶Department of Preclinical Development, Laboratoire Français du Fractionnement et des Biotechnologies, France; ††National Institute for Biological Standards and Control, UK, and ‡‡Center for Biologics Evaluation and Research, Food and Drug Administration, USA</mods:affiliation>
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<author><name sortKey="Cattaneo, M" sort="Cattaneo, M" uniqKey="Cattaneo M" first="M." last="Cattaneo">M. Cattaneo</name>
<affiliation><mods:affiliation>*Institute of Clinical Pathology and Medical Research, Australia; †Department of Pathology and Molecular Medicine, Richardson Laboratory, Queen's University, Canada; ‡Instituto de Investigaciones Hematológicas, Academia Nacional de Medicina, Argentina; §University of Milano, Ospedale San Paolo, Unit di Hematologica, Italy; ¶Department of Preclinical Development, Laboratoire Français du Fractionnement et des Biotechnologies, France; ††National Institute for Biological Standards and Control, UK, and ‡‡Center for Biologics Evaluation and Research, Food and Drug Administration, USA</mods:affiliation>
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<author><name sortKey="Mazurier, C" sort="Mazurier, C" uniqKey="Mazurier C" first="C." last="Mazurier">C. Mazurier</name>
<affiliation><mods:affiliation>*Institute of Clinical Pathology and Medical Research, Australia; †Department of Pathology and Molecular Medicine, Richardson Laboratory, Queen's University, Canada; ‡Instituto de Investigaciones Hematológicas, Academia Nacional de Medicina, Argentina; §University of Milano, Ospedale San Paolo, Unit di Hematologica, Italy; ¶Department of Preclinical Development, Laboratoire Français du Fractionnement et des Biotechnologies, France; ††National Institute for Biological Standards and Control, UK, and ‡‡Center for Biologics Evaluation and Research, Food and Drug Administration, USA</mods:affiliation>
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<author><name sortKey="Woods, A" sort="Woods, A" uniqKey="Woods A" first="A." last="Woods">A. Woods</name>
<affiliation><mods:affiliation>*Institute of Clinical Pathology and Medical Research, Australia; †Department of Pathology and Molecular Medicine, Richardson Laboratory, Queen's University, Canada; ‡Instituto de Investigaciones Hematológicas, Academia Nacional de Medicina, Argentina; §University of Milano, Ospedale San Paolo, Unit di Hematologica, Italy; ¶Department of Preclinical Development, Laboratoire Français du Fractionnement et des Biotechnologies, France; ††National Institute for Biological Standards and Control, UK, and ‡‡Center for Biologics Evaluation and Research, Food and Drug Administration, USA</mods:affiliation>
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<author><name sortKey="Meschengieser, S" sort="Meschengieser, S" uniqKey="Meschengieser S" first="S." last="Meschengieser">S. Meschengieser</name>
<affiliation><mods:affiliation>*Institute of Clinical Pathology and Medical Research, Australia; †Department of Pathology and Molecular Medicine, Richardson Laboratory, Queen's University, Canada; ‡Instituto de Investigaciones Hematológicas, Academia Nacional de Medicina, Argentina; §University of Milano, Ospedale San Paolo, Unit di Hematologica, Italy; ¶Department of Preclinical Development, Laboratoire Français du Fractionnement et des Biotechnologies, France; ††National Institute for Biological Standards and Control, UK, and ‡‡Center for Biologics Evaluation and Research, Food and Drug Administration, USA</mods:affiliation>
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<author><name sortKey="Blanco, A" sort="Blanco, A" uniqKey="Blanco A" first="A." last="Blanco">A. Blanco</name>
<affiliation><mods:affiliation>*Institute of Clinical Pathology and Medical Research, Australia; †Department of Pathology and Molecular Medicine, Richardson Laboratory, Queen's University, Canada; ‡Instituto de Investigaciones Hematológicas, Academia Nacional de Medicina, Argentina; §University of Milano, Ospedale San Paolo, Unit di Hematologica, Italy; ¶Department of Preclinical Development, Laboratoire Français du Fractionnement et des Biotechnologies, France; ††National Institute for Biological Standards and Control, UK, and ‡‡Center for Biologics Evaluation and Research, Food and Drug Administration, USA</mods:affiliation>
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<author><name sortKey="Kempfer, A C" sort="Kempfer, A C" uniqKey="Kempfer A" first="A. C." last="Kempfer">A. C. Kempfer</name>
<affiliation><mods:affiliation>*Institute of Clinical Pathology and Medical Research, Australia; †Department of Pathology and Molecular Medicine, Richardson Laboratory, Queen's University, Canada; ‡Instituto de Investigaciones Hematológicas, Academia Nacional de Medicina, Argentina; §University of Milano, Ospedale San Paolo, Unit di Hematologica, Italy; ¶Department of Preclinical Development, Laboratoire Français du Fractionnement et des Biotechnologies, France; ††National Institute for Biological Standards and Control, UK, and ‡‡Center for Biologics Evaluation and Research, Food and Drug Administration, USA</mods:affiliation>
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<author><name sortKey="Hubbard, A" sort="Hubbard, A" uniqKey="Hubbard A" first="A." last="Hubbard">A. Hubbard</name>
<affiliation><mods:affiliation>*Institute of Clinical Pathology and Medical Research, Australia; †Department of Pathology and Molecular Medicine, Richardson Laboratory, Queen's University, Canada; ‡Instituto de Investigaciones Hematológicas, Academia Nacional de Medicina, Argentina; §University of Milano, Ospedale San Paolo, Unit di Hematologica, Italy; ¶Department of Preclinical Development, Laboratoire Français du Fractionnement et des Biotechnologies, France; ††National Institute for Biological Standards and Control, UK, and ‡‡Center for Biologics Evaluation and Research, Food and Drug Administration, USA</mods:affiliation>
</affiliation>
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<author><name sortKey="Chang, A" sort="Chang, A" uniqKey="Chang A" first="A." last="Chang">A. Chang</name>
<affiliation><mods:affiliation>*Institute of Clinical Pathology and Medical Research, Australia; †Department of Pathology and Molecular Medicine, Richardson Laboratory, Queen's University, Canada; ‡Instituto de Investigaciones Hematológicas, Academia Nacional de Medicina, Argentina; §University of Milano, Ospedale San Paolo, Unit di Hematologica, Italy; ¶Department of Preclinical Development, Laboratoire Français du Fractionnement et des Biotechnologies, France; ††National Institute for Biological Standards and Control, UK, and ‡‡Center for Biologics Evaluation and Research, Food and Drug Administration, USA</mods:affiliation>
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<term>Collagen binding</term>
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<term>Favaloro</term>
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<term>Fviii level</term>
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<term>Possible type</term>
<term>Quantitative defect</term>
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<front><div type="abstract">Summary. von Willebrand disease is the most common inherited bleeding disorder in humans. VWD can be classified into three major types, designated Types 1, 2 and 3; Type 2 can be further separated into subtypes 2A, 2B, 2M and 2N. The diagnosis of VWD requires a personal and family history of bleeding and confirmation by laboratory analysis. Although Types 2 and 3 are relatively straightforward to diagnose, there may be a risk of overdiagnosis of Type 1 because of an overlap within the normal range. We also report on the clinical profile and diagnosis of VWD in a South American cohort of patients and on the in vitro characteristics of some factor concentrates available for treatment of VWD.</div>
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<json:item><name>D. Lillicrap</name>
<affiliations><json:string>*Institute of Clinical Pathology and Medical Research, Australia; †Department of Pathology and Molecular Medicine, Richardson Laboratory, Queen's University, Canada; ‡Instituto de Investigaciones Hematológicas, Academia Nacional de Medicina, Argentina; §University of Milano, Ospedale San Paolo, Unit di Hematologica, Italy; ¶Department of Preclinical Development, Laboratoire Français du Fractionnement et des Biotechnologies, France; ††National Institute for Biological Standards and Control, UK, and ‡‡Center for Biologics Evaluation and Research, Food and Drug Administration, USA</json:string>
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<json:item><name>M. A. Lazzari</name>
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<json:item><name>M. Cattaneo</name>
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<correspondenceTo>EJ Favaloro, Institute of Clinical Pathology and Medical Research (ICPMR), Westmead Hospital, Westmead NSW, 2145, Australia.
Tel.: +(612) 9845 6618; fax: +(612) 9689 2331;
e‐mail: <email>emmanuel@icpmr.wsahs.nsw.gov.au</email>
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*Institute of Clinical Pathology and Medical Research, Australia
;
†Department of Pathology and Molecular Medicine, Richardson Laboratory, Queen's University, Canada
;
‡Instituto de Investigaciones Hematológicas, Academia Nacional de Medicina, Argentina
;
§University of Milano, Ospedale San Paolo, Unit di Hematologica, Italy
;
¶Department of Preclinical Development, Laboratoire Français du Fractionnement et des Biotechnologies, France
;
††National Institute for Biological Standards and Control, UK
, and
‡‡Center for Biologics Evaluation and Research, Food and Drug Administration, USA</unparsedAffiliation>
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<keywordGroup xml:lang="en"><keyword xml:id="k1">factor concentrate</keyword>
<keyword xml:id="k2">diagnosis</keyword>
<keyword xml:id="k3">treatment</keyword>
<keyword xml:id="k4">von Willebrand disease</keyword>
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<abstractGroup><abstract type="main" xml:lang="en"><p><b>Summary. </b>
von Willebrand disease is the most common inherited bleeding disorder in humans. VWD can be classified into three major types, designated Types 1, 2 and 3; Type 2 can be further separated into subtypes 2A, 2B, 2M and 2N. The diagnosis of VWD requires a personal and family history of bleeding and confirmation by laboratory analysis. Although Types 2 and 3 are relatively straightforward to diagnose, there may be a risk of overdiagnosis of Type 1 because of an overlap within the normal range. We also report on the clinical profile and diagnosis of VWD in a South American cohort of patients and on the <i>in vitro</i>
characteristics of some factor concentrates available for treatment of VWD.</p>
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<mods version="3.6"><titleInfo lang="en"><title>von Willebrand disease: laboratory aspects of diagnosis and treatment</title>
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<titleInfo type="abbreviated" lang="en"><title>VWD: LABORATORY ASPECTS TO DIAGNOSIS AND TREATMENT</title>
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<affiliation>*Institute of Clinical Pathology and Medical Research, Australia; †Department of Pathology and Molecular Medicine, Richardson Laboratory, Queen's University, Canada; ‡Instituto de Investigaciones Hematológicas, Academia Nacional de Medicina, Argentina; §University of Milano, Ospedale San Paolo, Unit di Hematologica, Italy; ¶Department of Preclinical Development, Laboratoire Français du Fractionnement et des Biotechnologies, France; ††National Institute for Biological Standards and Control, UK, and ‡‡Center for Biologics Evaluation and Research, Food and Drug Administration, USA</affiliation>
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<namePart type="family">Lillicrap</namePart>
<affiliation>*Institute of Clinical Pathology and Medical Research, Australia; †Department of Pathology and Molecular Medicine, Richardson Laboratory, Queen's University, Canada; ‡Instituto de Investigaciones Hematológicas, Academia Nacional de Medicina, Argentina; §University of Milano, Ospedale San Paolo, Unit di Hematologica, Italy; ¶Department of Preclinical Development, Laboratoire Français du Fractionnement et des Biotechnologies, France; ††National Institute for Biological Standards and Control, UK, and ‡‡Center for Biologics Evaluation and Research, Food and Drug Administration, USA</affiliation>
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<affiliation>*Institute of Clinical Pathology and Medical Research, Australia; †Department of Pathology and Molecular Medicine, Richardson Laboratory, Queen's University, Canada; ‡Instituto de Investigaciones Hematológicas, Academia Nacional de Medicina, Argentina; §University of Milano, Ospedale San Paolo, Unit di Hematologica, Italy; ¶Department of Preclinical Development, Laboratoire Français du Fractionnement et des Biotechnologies, France; ††National Institute for Biological Standards and Control, UK, and ‡‡Center for Biologics Evaluation and Research, Food and Drug Administration, USA</affiliation>
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<affiliation>*Institute of Clinical Pathology and Medical Research, Australia; †Department of Pathology and Molecular Medicine, Richardson Laboratory, Queen's University, Canada; ‡Instituto de Investigaciones Hematológicas, Academia Nacional de Medicina, Argentina; §University of Milano, Ospedale San Paolo, Unit di Hematologica, Italy; ¶Department of Preclinical Development, Laboratoire Français du Fractionnement et des Biotechnologies, France; ††National Institute for Biological Standards and Control, UK, and ‡‡Center for Biologics Evaluation and Research, Food and Drug Administration, USA</affiliation>
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<abstract>Summary. von Willebrand disease is the most common inherited bleeding disorder in humans. VWD can be classified into three major types, designated Types 1, 2 and 3; Type 2 can be further separated into subtypes 2A, 2B, 2M and 2N. The diagnosis of VWD requires a personal and family history of bleeding and confirmation by laboratory analysis. Although Types 2 and 3 are relatively straightforward to diagnose, there may be a risk of overdiagnosis of Type 1 because of an overlap within the normal range. We also report on the clinical profile and diagnosis of VWD in a South American cohort of patients and on the in vitro characteristics of some factor concentrates available for treatment of VWD.</abstract>
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