Changing prognosis in paroxysmal nocturnal haemoglobinuria disease subcategories: an analysis of the International PNH Registry.
Identifieur interne : 002244 ( Main/Curation ); précédent : 002243; suivant : 002245Changing prognosis in paroxysmal nocturnal haemoglobinuria disease subcategories: an analysis of the International PNH Registry.
Auteurs : G. Socié [France] ; H. Schrezenmeier [Allemagne] ; P. Muus [Pays-Bas] ; I. Lisukov [Russie] ; A. Röth [Allemagne] ; A. Kulasekararaj [Royaume-Uni] ; J W Lee [Corée du Sud] ; D. Araten [États-Unis] ; A. Hill [Royaume-Uni] ; R. Brodsky [États-Unis] ; A. Urbano-Ispizua [Espagne] ; J. Szer [Australie] ; A. Wilson [États-Unis] ; P. Hillmen [Royaume-Uni]Source :
- Internal medicine journal [ 1445-5994 ] ; 2016.
Descripteurs français
- KwdFr :
- Adulte, Adulte d'âge moyen, Analyse de survie, Analyse multivariée, Anticorps monoclonaux humanisés (usage thérapeutique), Anémie aplasique (épidémiologie), Cause de décès, Enregistrements, Facteurs de risque, Femelle, France, Humains, Hémoglobinurie paroxystique (), Hémoglobinurie paroxystique (mortalité), Incidence, Mâle, Pronostic, Thrombose (épidémiologie), Transfusion d'érythrocytes, Études rétrospectives.
- MESH :
- mortalité : Hémoglobinurie paroxystique.
- usage thérapeutique : Anticorps monoclonaux humanisés.
- épidémiologie : Anémie aplasique, Thrombose.
- Adulte, Adulte d'âge moyen, Analyse de survie, Analyse multivariée, Cause de décès, Enregistrements, Facteurs de risque, Femelle, France, Humains, Hémoglobinurie paroxystique, Incidence, Mâle, Pronostic, Transfusion d'érythrocytes, Études rétrospectives.
English descriptors
- KwdEn :
- Adult, Anemia, Aplastic (epidemiology), Antibodies, Monoclonal, Humanized (therapeutic use), Cause of Death, Erythrocyte Transfusion, Female, France, Hemoglobinuria, Paroxysmal (classification), Hemoglobinuria, Paroxysmal (mortality), Hemoglobinuria, Paroxysmal (therapy), Humans, Incidence, Male, Middle Aged, Multivariate Analysis, Prognosis, Registries, Retrospective Studies, Risk Factors, Survival Analysis, Thrombosis (epidemiology).
- MESH :
- chemical , therapeutic use : Antibodies, Monoclonal, Humanized.
- classification : Hemoglobinuria, Paroxysmal.
- epidemiology : Anemia, Aplastic, Thrombosis.
- mortality : Hemoglobinuria, Paroxysmal.
- therapy : Hemoglobinuria, Paroxysmal.
- Adult, Cause of Death, Erythrocyte Transfusion, Female, France, Humans, Incidence, Male, Middle Aged, Multivariate Analysis, Prognosis, Registries, Retrospective Studies, Risk Factors, Survival Analysis.
Abstract
Paroxysmal nocturnal haemoglobinuria (PNH) is a rare disease. Although much progress has been made in the understanding of the pathophysiology of the disease, far less is known with respect to the clinical outcomes of patients with PNH. Few retrospective studies provide survival estimates, and even fewer have explored the clinical heterogeneity of the disease. Haemolytic and aplastic anaemia (AA) forms of the disease have been recognised as main disease categories, with the haemolytic form being associated with the worst prognosis by the largest studied cohort some years ago.
DOI: 10.1111/imj.13160
PubMed: 27305361
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Socié</name><affiliation wicri:level="3"><nlm:affiliation>Hematology Transplantation, AP-HP, Hospital Saint Louis, University Paris VII Denis Diderot and Inserm UMR 1160, Paris, France. gerard.socie@sls.aphp.fr.</nlm:affiliation><country xml:lang="fr">France</country><wicri:regionArea>Hematology Transplantation, AP-HP, Hospital Saint Louis, University Paris VII Denis Diderot and Inserm UMR 1160, Paris</wicri:regionArea><placeName><region type="region">Île-de-France</region><region type="old region">Île-de-France</region><settlement type="city">Paris</settlement></placeName></affiliation></author><author><name sortKey="Schrezenmeier, H" sort="Schrezenmeier, H" uniqKey="Schrezenmeier H" first="H" last="Schrezenmeier">H. 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Socié</name><affiliation wicri:level="3"><nlm:affiliation>Hematology Transplantation, AP-HP, Hospital Saint Louis, University Paris VII Denis Diderot and Inserm UMR 1160, Paris, France. gerard.socie@sls.aphp.fr.</nlm:affiliation><country xml:lang="fr">France</country><wicri:regionArea>Hematology Transplantation, AP-HP, Hospital Saint Louis, University Paris VII Denis Diderot and Inserm UMR 1160, Paris</wicri:regionArea><placeName><region type="region">Île-de-France</region><region type="old region">Île-de-France</region><settlement type="city">Paris</settlement></placeName></affiliation></author><author><name sortKey="Schrezenmeier, H" sort="Schrezenmeier, H" uniqKey="Schrezenmeier H" first="H" last="Schrezenmeier">H. Schrezenmeier</name><affiliation wicri:level="3"><nlm:affiliation>Institute of Transfusion Medicine, University of Ulm Ulm and Institute of Clinical Transfusion Medicine and Immunogenetics, German Red Cross Blood Transfusion Service Baden-Württemberg-Hessen and University Hospital Ulm, Ulm, Germany.</nlm:affiliation><country xml:lang="fr">Allemagne</country><wicri:regionArea>Institute of Transfusion Medicine, University of Ulm Ulm and Institute of Clinical Transfusion Medicine and Immunogenetics, German Red Cross Blood Transfusion Service Baden-Württemberg-Hessen and University Hospital Ulm, Ulm</wicri:regionArea><placeName><region type="land" nuts="1">Bade-Wurtemberg</region><region type="district" nuts="2">District de Tübingen</region><settlement type="city">Ulm</settlement></placeName></affiliation></author><author><name sortKey="Muus, P" sort="Muus, P" uniqKey="Muus P" first="P" last="Muus">P. Muus</name><affiliation wicri:level="3"><nlm:affiliation>Department of Hematology, Radboud UMC, Nijmegen, the Netherlands.</nlm:affiliation><country xml:lang="fr" wicri:curation="lc">Pays-Bas</country><wicri:regionArea>Department of Hematology, Radboud UMC, Nijmegen</wicri:regionArea><placeName><settlement type="city">Nimègue</settlement><region type="province" nuts="2">Gueldre</region></placeName></affiliation></author><author><name sortKey="Lisukov, I" sort="Lisukov, I" uniqKey="Lisukov I" first="I" last="Lisukov">I. Lisukov</name><affiliation wicri:level="1"><nlm:affiliation>Institution of Children Hematology and Transplantation, St. Petersburg, Russia.</nlm:affiliation><country xml:lang="fr">Russie</country><wicri:regionArea>Institution of Children Hematology and Transplantation, St. Petersburg</wicri:regionArea><wicri:noRegion>St. Petersburg</wicri:noRegion></affiliation></author><author><name sortKey="Roth, A" sort="Roth, A" uniqKey="Roth A" first="A" last="Röth">A. Röth</name><affiliation wicri:level="1"><nlm:affiliation>University Hospital Essen, Essen, Germany.</nlm:affiliation><country xml:lang="fr">Allemagne</country><wicri:regionArea>University Hospital Essen, Essen</wicri:regionArea><wicri:noRegion>Essen</wicri:noRegion><wicri:noRegion>Essen</wicri:noRegion><wicri:noRegion>Essen</wicri:noRegion></affiliation></author><author><name sortKey="Kulasekararaj, A" sort="Kulasekararaj, A" uniqKey="Kulasekararaj A" first="A" last="Kulasekararaj">A. Kulasekararaj</name><affiliation wicri:level="3"><nlm:affiliation>Kings College Hospital, London, UK.</nlm:affiliation><country xml:lang="fr">Royaume-Uni</country><wicri:regionArea>Kings College Hospital, London</wicri:regionArea><placeName><settlement type="city">Londres</settlement><region type="country">Angleterre</region><region type="région" nuts="1">Grand Londres</region></placeName></affiliation></author><author><name sortKey="Lee, J W" sort="Lee, J W" uniqKey="Lee J" first="J W" last="Lee">J W Lee</name><affiliation wicri:level="3"><nlm:affiliation>Seoul St. Mary's Hospital, Seoul, Korea.</nlm:affiliation><country xml:lang="fr">Corée du Sud</country><wicri:regionArea>Seoul St. Mary's Hospital, Seoul</wicri:regionArea><placeName><settlement type="city">Séoul</settlement><region type="capital">Région capitale de Séoul</region></placeName></affiliation></author><author><name sortKey="Araten, D" sort="Araten, D" uniqKey="Araten D" first="D" last="Araten">D. Araten</name><affiliation wicri:level="1"><nlm:affiliation>Laura and Isaac Perlmutter Cancer Center, NYU Langone Medical Center, New York VA Medical Center, USA.</nlm:affiliation><country xml:lang="fr">États-Unis</country><wicri:regionArea>Laura and Isaac Perlmutter Cancer Center, NYU Langone Medical Center, New York VA Medical Center</wicri:regionArea><wicri:noRegion>New York VA Medical Center</wicri:noRegion></affiliation></author><author><name sortKey="Hill, A" sort="Hill, A" uniqKey="Hill A" first="A" last="Hill">A. Hill</name><affiliation wicri:level="1"><nlm:affiliation>St James's University Hospital, Leeds, UK.</nlm:affiliation><country xml:lang="fr">Royaume-Uni</country><wicri:regionArea>St James's University Hospital, Leeds</wicri:regionArea><wicri:noRegion>Leeds</wicri:noRegion></affiliation></author><author><name sortKey="Brodsky, R" sort="Brodsky, R" uniqKey="Brodsky R" first="R" last="Brodsky">R. Brodsky</name><affiliation wicri:level="1"><nlm:affiliation>Johns Hopkins University Medical Center, USA.</nlm:affiliation><country xml:lang="fr">États-Unis</country><wicri:regionArea>Johns Hopkins University Medical Center</wicri:regionArea></affiliation></author><author><name sortKey="Urbano Ispizua, A" sort="Urbano Ispizua, A" uniqKey="Urbano Ispizua A" first="A" last="Urbano-Ispizua">A. Urbano-Ispizua</name><affiliation wicri:level="3"><nlm:affiliation>Hospital Clínic Barcelona, Barcelona, Spain.</nlm:affiliation><country xml:lang="fr">Espagne</country><wicri:regionArea>Hospital Clínic Barcelona, Barcelona</wicri:regionArea><placeName><settlement type="city">Barcelone</settlement><region nuts="2" type="region">Catalogne</region></placeName></affiliation></author><author><name sortKey="Szer, J" sort="Szer, J" uniqKey="Szer J" first="J" last="Szer">J. Szer</name><affiliation wicri:level="1"><nlm:affiliation>Royal Melbourne Hospital, Melbourne, Victoria, Australia.</nlm:affiliation><country xml:lang="fr">Australie</country><wicri:regionArea>Royal Melbourne Hospital, Melbourne, Victoria</wicri:regionArea><wicri:noRegion>Victoria</wicri:noRegion></affiliation></author><author><name sortKey="Wilson, A" sort="Wilson, A" uniqKey="Wilson A" first="A" last="Wilson">A. Wilson</name><affiliation wicri:level="2"><nlm:affiliation>Alexion Pharmaceuticals, New Haven, Connecticut, USA.</nlm:affiliation><country xml:lang="fr">États-Unis</country><wicri:regionArea>Alexion Pharmaceuticals, New Haven, Connecticut</wicri:regionArea><placeName><region type="state">Connecticut</region></placeName></affiliation></author><author><name sortKey="Hillmen, P" sort="Hillmen, P" uniqKey="Hillmen P" first="P" last="Hillmen">P. Hillmen</name><affiliation wicri:level="1"><nlm:affiliation>St James's University Hospital, Leeds, UK.</nlm:affiliation><country xml:lang="fr">Royaume-Uni</country><wicri:regionArea>St James's University Hospital, Leeds</wicri:regionArea><wicri:noRegion>Leeds</wicri:noRegion></affiliation></author></analytic><series><title level="j">Internal medicine journal</title><idno type="eISSN">1445-5994</idno><imprint><date when="2016" type="published">2016</date></imprint></series></biblStruct></sourceDesc></fileDesc><profileDesc><textClass><keywords scheme="KwdEn" xml:lang="en"><term>Adult</term><term>Anemia, Aplastic (epidemiology)</term><term>Antibodies, Monoclonal, Humanized (therapeutic use)</term><term>Cause of Death</term><term>Erythrocyte Transfusion</term><term>Female</term><term>France</term><term>Hemoglobinuria, Paroxysmal (classification)</term><term>Hemoglobinuria, Paroxysmal (mortality)</term><term>Hemoglobinuria, Paroxysmal (therapy)</term><term>Humans</term><term>Incidence</term><term>Male</term><term>Middle Aged</term><term>Multivariate Analysis</term><term>Prognosis</term><term>Registries</term><term>Retrospective Studies</term><term>Risk Factors</term><term>Survival Analysis</term><term>Thrombosis (epidemiology)</term></keywords><keywords scheme="KwdFr" xml:lang="fr"><term>Adulte</term><term>Adulte d'âge moyen</term><term>Analyse de survie</term><term>Analyse multivariée</term><term>Anticorps monoclonaux humanisés (usage thérapeutique)</term><term>Anémie aplasique (épidémiologie)</term><term>Cause de décès</term><term>Enregistrements</term><term>Facteurs de risque</term><term>Femelle</term><term>France</term><term>Humains</term><term>Hémoglobinurie paroxystique ()</term><term>Hémoglobinurie paroxystique (mortalité)</term><term>Incidence</term><term>Mâle</term><term>Pronostic</term><term>Thrombose (épidémiologie)</term><term>Transfusion d'érythrocytes</term><term>Études rétrospectives</term></keywords><keywords scheme="MESH" type="chemical" qualifier="therapeutic use" xml:lang="en"><term>Antibodies, Monoclonal, Humanized</term></keywords><keywords scheme="MESH" qualifier="classification" xml:lang="en"><term>Hemoglobinuria, Paroxysmal</term></keywords><keywords scheme="MESH" qualifier="epidemiology" xml:lang="en"><term>Anemia, Aplastic</term><term>Thrombosis</term></keywords><keywords scheme="MESH" qualifier="mortality" xml:lang="en"><term>Hemoglobinuria, Paroxysmal</term></keywords><keywords scheme="MESH" qualifier="mortalité" xml:lang="fr"><term>Hémoglobinurie paroxystique</term></keywords><keywords scheme="MESH" qualifier="therapy" xml:lang="en"><term>Hemoglobinuria, Paroxysmal</term></keywords><keywords scheme="MESH" qualifier="usage thérapeutique" xml:lang="fr"><term>Anticorps monoclonaux humanisés</term></keywords><keywords scheme="MESH" qualifier="épidémiologie" xml:lang="fr"><term>Anémie aplasique</term><term>Thrombose</term></keywords><keywords scheme="MESH" xml:lang="en"><term>Adult</term><term>Cause of Death</term><term>Erythrocyte Transfusion</term><term>Female</term><term>France</term><term>Humans</term><term>Incidence</term><term>Male</term><term>Middle Aged</term><term>Multivariate Analysis</term><term>Prognosis</term><term>Registries</term><term>Retrospective Studies</term><term>Risk Factors</term><term>Survival Analysis</term></keywords><keywords scheme="MESH" xml:lang="fr"><term>Adulte</term><term>Adulte d'âge moyen</term><term>Analyse de survie</term><term>Analyse multivariée</term><term>Cause de décès</term><term>Enregistrements</term><term>Facteurs de risque</term><term>Femelle</term><term>France</term><term>Humains</term><term>Hémoglobinurie paroxystique</term><term>Incidence</term><term>Mâle</term><term>Pronostic</term><term>Transfusion d'érythrocytes</term><term>Études rétrospectives</term></keywords></textClass></profileDesc></teiHeader><front><div type="abstract" xml:lang="en">Paroxysmal nocturnal haemoglobinuria (PNH) is a rare disease. Although much progress has been made in the understanding of the pathophysiology of the disease, far less is known with respect to the clinical outcomes of patients with PNH. Few retrospective studies provide survival estimates, and even fewer have explored the clinical heterogeneity of the disease. Haemolytic and aplastic anaemia (AA) forms of the disease have been recognised as main disease categories, with the haemolytic form being associated with the worst prognosis by the largest studied cohort some years ago.</div></front></TEI></record>Pour manipuler ce document sous Unix (Dilib)
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