Durable responses to imatinib in patients with PDGFRB fusion gene-positive and BCR-ABL-negative chronic myeloproliferative disorders
Identifieur interne : 009631 ( Main/Exploration ); précédent : 009630; suivant : 009632Durable responses to imatinib in patients with PDGFRB fusion gene-positive and BCR-ABL-negative chronic myeloproliferative disorders
Auteurs : Marianna David [Hongrie] ; Nicholas C. P. Cross [Royaume-Uni] ; Sonja Burgstaller [Autriche] ; Andrew Chase [Royaume-Uni] ; Claire Curtis [Royaume-Uni] ; Raymond Dang [Royaume-Uni] ; Martine Gardembas [France] ; John M. Goldman [Royaume-Uni] ; Francis Grand [Royaume-Uni] ; George Hughes [Royaume-Uni] ; Francoise Huguet [France] ; Louise Lavender [Royaume-Uni] ; Grant A. Mcarthur [Australie] ; Francois X. Mahon [France] ; Giorgio Massimini [Suisse] ; Junia Melo [Royaume-Uni] ; Philippe Rousselot [France] ; Robin J. Russell-Jones [Royaume-Uni] ; John F. Seymour [Australie] ; Graeme Smith [Royaume-Uni] ; Alastair Stark [Royaume-Uni] ; Katherine Waghorn [Royaume-Uni] ; Zariana Nikolova [Suisse] ; Jane F. Apperley [Royaume-Uni]Source :
- Blood [ 0006-4971 ] ; 2007.
Descripteurs français
- Pascal (Inist)
- Imatinib, Protein-tyrosine kinase, Inhibiteur enzyme, Myéloprolifératif syndrome, Homme, Gène hybride, Translocation chromosomique, Chromosome C9 anormal, Chromosome G22 anormal, Chromosome Ph1, Protein kinase, Chronique, Hématologie, Anticancéreux, Gène bcr, Gène abl, Récepteur PDGFRB, Protéine bcr-abl.
- Wicri :
- topic : Homme.
English descriptors
- KwdEn :
- Abnormal chromosome C9, Abnormal chromosome G22, Antineoplastic agent, Bcr-abl protein, Chromosome translocation, Chronic, Enzyme inhibitor, Hematology, Human, Hybrid gene, Imatinib, Myeloproliferative syndrome, Philadelphia chromosome, Platelet derived growth factor receptor beta, Protein kinase, Protein-tyrosine kinase.
Abstract
Fusion genes derived from the platelet-derived growth factor receptor beta (PDGFRB) or alpha (PDGFRA) play an important role in the pathogenesis of BCR-ABL-negative chronic myeloproliferative disorders (CMPDs). These fusion genes encode constitutively activated receptor tyrosine kinases that can be inhibited by imatinib. Twelve patients with BCR-ABL-negative CMPDs and reciprocal translocations involving PDGFRB received imatinib for a median of 47 months (range, 0.1-60 months). Eleven had prompt responses with normalization of peripheral-blood cell counts and disappearance of eosinophilia; 10 had complete resolution of cytogenetic abnormalities and decrease or disappearance of fusion transcripts as measured by reverse transcriptase-polymerase chain reaction (RT-PCR). Updates were sought from 8 further patients previously described in the literature; prompt responses were described in 7 and persist in 6. Our data show that durable hematologic and cytogenetic responses are achieved with imatinib in patients with PDGFRB fusion-positive, BCR-ABL-negative CMPDs.
Affiliations:
- Australie, Autriche, France, Hongrie, Royaume-Uni, Suisse
- Angleterre, Aquitaine, Grand Londres, Midi-Pyrénées, Nouvelle-Aquitaine, Occitanie (région administrative), Pays de la Loire, Victoria (État), Île-de-France
- Angers, Bordeaux, Londres, Melbourne, Paris, Toulouse
Links toward previous steps (curation, corpus...)
- to stream PascalFrancis, to step Corpus: 003C34
- to stream PascalFrancis, to step Curation: 002454
- to stream PascalFrancis, to step Checkpoint: 003954
- to stream Main, to step Merge: 00A066
- to stream Main, to step Curation: 009631
Le document en format XML
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Goldman</name><affiliation wicri:level="3"><inist:fA14 i1="06"><s1>Department of Haematology, Faculty of Medicine, Imperial College</s1><s2>London</s2><s3>GBR</s3><sZ>8 aut.</sZ><sZ>16 aut.</sZ><sZ>24 aut.</sZ></inist:fA14><country>Royaume-Uni</country><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="Grand, Francis" sort="Grand, Francis" uniqKey="Grand F" first="Francis" last="Grand">Francis Grand</name><affiliation wicri:level="1"><inist:fA14 i1="02"><s1>Wessex Regional Genetics Laboratory, Salisbury District Hospital</s1><s2>Salisbury</s2><s3>GBR</s3><sZ>2 aut.</sZ><sZ>4 aut.</sZ><sZ>5 aut.</sZ><sZ>9 aut.</sZ><sZ>22 aut.</sZ></inist:fA14><country>Royaume-Uni</country><wicri:noRegion>Salisbury</wicri:noRegion></affiliation></author><author><name sortKey="Hughes, George" sort="Hughes, George" uniqKey="Hughes G" first="George" last="Hughes">George Hughes</name><affiliation wicri:level="3"><inist:fA14 i1="07"><s1>Department of Haematology, West Middlesex Hospital</s1><s2>London</s2><s3>GBR</s3><sZ>10 aut.</sZ></inist:fA14><country>Royaume-Uni</country><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="Huguet, Francoise" sort="Huguet, Francoise" uniqKey="Huguet F" first="Francoise" last="Huguet">Francoise Huguet</name><affiliation wicri:level="3"><inist:fA14 i1="08"><s1>Department of Haematology, Hopital de Purpan</s1><s2>Toulouse</s2><s3>FRA</s3><sZ>11 aut.</sZ></inist:fA14><country>France</country><placeName><region type="region">Occitanie (région administrative)</region><region type="old region">Midi-Pyrénées</region><settlement type="city">Toulouse</settlement></placeName></affiliation></author><author><name sortKey="Lavender, Louise" sort="Lavender, Louise" uniqKey="Lavender L" first="Louise" last="Lavender">Louise Lavender</name><affiliation wicri:level="1"><inist:fA14 i1="09"><s1>Molecular Pathology Unit, Southampton General Hospital</s1><s2>Southampton</s2><s3>GBR</s3><sZ>12 aut.</sZ></inist:fA14><country>Royaume-Uni</country><wicri:noRegion>Southampton</wicri:noRegion></affiliation></author><author><name sortKey="Mcarthur, Grant A" sort="Mcarthur, Grant A" uniqKey="Mcarthur G" first="Grant A." last="Mcarthur">Grant A. Mcarthur</name><affiliation wicri:level="3"><inist:fA14 i1="10"><s1>Department of Haematology, Peter MacCallum Cancer Centre</s1><s2>Melbourne</s2><s3>AUS</s3><sZ>13 aut.</sZ><sZ>19 aut.</sZ></inist:fA14><country>Australie</country><placeName><settlement type="city">Melbourne</settlement><region type="état">Victoria (État)</region></placeName></affiliation></author><author><name sortKey="Mahon, Francois X" sort="Mahon, Francois X" uniqKey="Mahon F" first="Francois X." last="Mahon">Francois X. Mahon</name><affiliation wicri:level="3"><inist:fA14 i1="11"><s1>Laboratory of normal and pathological haematology, University Victor Ségalen</s1><s2>Bordeaux</s2><s3>FRA</s3><sZ>14 aut.</sZ></inist:fA14><country>France</country><placeName><region type="region">Nouvelle-Aquitaine</region><region type="old region">Aquitaine</region><settlement type="city">Bordeaux</settlement></placeName></affiliation></author><author><name sortKey="Massimini, Giorgio" sort="Massimini, Giorgio" uniqKey="Massimini G" first="Giorgio" last="Massimini">Giorgio Massimini</name><affiliation wicri:level="1"><inist:fA14 i1="12"><s1>Novartis Oncology</s1><s2>Basel</s2><s3>CHE</s3><sZ>15 aut.</sZ><sZ>23 aut.</sZ></inist:fA14><country>Suisse</country><wicri:noRegion>Novartis Oncology</wicri:noRegion></affiliation></author><author><name sortKey="Melo, Junia" sort="Melo, Junia" uniqKey="Melo J" first="Junia" last="Melo">Junia Melo</name><affiliation wicri:level="3"><inist:fA14 i1="06"><s1>Department of Haematology, Faculty of Medicine, Imperial College</s1><s2>London</s2><s3>GBR</s3><sZ>8 aut.</sZ><sZ>16 aut.</sZ><sZ>24 aut.</sZ></inist:fA14><country>Royaume-Uni</country><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="Rousselot, Philippe" sort="Rousselot, Philippe" uniqKey="Rousselot P" first="Philippe" last="Rousselot">Philippe Rousselot</name><affiliation wicri:level="3"><inist:fA14 i1="13"><s1>Department of Haematology, Hopital St Louis</s1><s2>Paris</s2><s3>FRA</s3><sZ>17 aut.</sZ></inist:fA14><country>France</country><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="Russell Jones, Robin J" sort="Russell Jones, Robin J" uniqKey="Russell Jones R" first="Robin J." last="Russell-Jones">Robin J. Russell-Jones</name><affiliation wicri:level="3"><inist:fA14 i1="14"><s1>Skin Tumour Unit, St John's Institute of Dermatology, St Thomas' Hospital, Lambeth Palace Road</s1><s2>London</s2><s3>GBR</s3><sZ>18 aut.</sZ></inist:fA14><country>Royaume-Uni</country><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="Seymour, John F" sort="Seymour, John F" uniqKey="Seymour J" first="John F." last="Seymour">John F. Seymour</name><affiliation wicri:level="3"><inist:fA14 i1="10"><s1>Department of Haematology, Peter MacCallum Cancer Centre</s1><s2>Melbourne</s2><s3>AUS</s3><sZ>13 aut.</sZ><sZ>19 aut.</sZ></inist:fA14><country>Australie</country><placeName><settlement type="city">Melbourne</settlement><region type="état">Victoria (État)</region></placeName></affiliation></author><author><name sortKey="Smith, Graeme" sort="Smith, Graeme" uniqKey="Smith G" first="Graeme" last="Smith">Graeme Smith</name><affiliation wicri:level="1"><inist:fA14 i1="15"><s1>Department of Haematology, Leeds General Infirmary</s1><s2>Leeds</s2><s3>GBR</s3><sZ>20 aut.</sZ></inist:fA14><country>Royaume-Uni</country><wicri:noRegion>Leeds</wicri:noRegion></affiliation></author><author><name sortKey="Stark, Alastair" sort="Stark, Alastair" uniqKey="Stark A" first="Alastair" last="Stark">Alastair Stark</name><affiliation wicri:level="1"><inist:fA14 i1="04"><s1>Department of Haematology, Dumfries General Infirmary</s1><s2>Dumfries, Scotland</s2><s3>GBR</s3><sZ>6 aut.</sZ><sZ>21 aut.</sZ></inist:fA14><country>Royaume-Uni</country><wicri:noRegion>Dumfries, Scotland</wicri:noRegion></affiliation></author><author><name sortKey="Waghorn, Katherine" sort="Waghorn, Katherine" uniqKey="Waghorn K" first="Katherine" last="Waghorn">Katherine Waghorn</name><affiliation wicri:level="1"><inist:fA14 i1="02"><s1>Wessex Regional Genetics Laboratory, Salisbury District Hospital</s1><s2>Salisbury</s2><s3>GBR</s3><sZ>2 aut.</sZ><sZ>4 aut.</sZ><sZ>5 aut.</sZ><sZ>9 aut.</sZ><sZ>22 aut.</sZ></inist:fA14><country>Royaume-Uni</country><wicri:noRegion>Salisbury</wicri:noRegion></affiliation></author><author><name sortKey="Nikolova, Zariana" sort="Nikolova, Zariana" uniqKey="Nikolova Z" first="Zariana" last="Nikolova">Zariana Nikolova</name><affiliation wicri:level="1"><inist:fA14 i1="12"><s1>Novartis Oncology</s1><s2>Basel</s2><s3>CHE</s3><sZ>15 aut.</sZ><sZ>23 aut.</sZ></inist:fA14><country>Suisse</country><wicri:noRegion>Novartis Oncology</wicri:noRegion></affiliation></author><author><name sortKey="Apperley, Jane F" sort="Apperley, Jane F" uniqKey="Apperley J" first="Jane F." last="Apperley">Jane F. Apperley</name><affiliation wicri:level="3"><inist:fA14 i1="06"><s1>Department of Haematology, Faculty of Medicine, Imperial College</s1><s2>London</s2><s3>GBR</s3><sZ>8 aut.</sZ><sZ>16 aut.</sZ><sZ>24 aut.</sZ></inist:fA14><country>Royaume-Uni</country><placeName><settlement type="city">Londres</settlement><region type="country">Angleterre</region><region type="région" nuts="1">Grand Londres</region></placeName></affiliation></author></titleStmt><publicationStmt><idno type="wicri:source">INIST</idno><idno type="inist">07-0247422</idno><date when="2007">2007</date><idno type="stanalyst">PASCAL 07-0247422 INIST</idno><idno type="RBID">Pascal:07-0247422</idno><idno type="wicri:Area/PascalFrancis/Corpus">003C34</idno><idno type="wicri:Area/PascalFrancis/Curation">002454</idno><idno type="wicri:Area/PascalFrancis/Checkpoint">003954</idno><idno type="wicri:explorRef" wicri:stream="PascalFrancis" wicri:step="Checkpoint">003954</idno><idno type="wicri:doubleKey">0006-4971:2007:David M:durable:responses:to</idno><idno type="wicri:Area/Main/Merge">00A066</idno><idno type="wicri:Area/Main/Curation">009631</idno><idno type="wicri:Area/Main/Exploration">009631</idno></publicationStmt><sourceDesc><biblStruct><analytic><title xml:lang="en" level="a">Durable responses to imatinib in patients with PDGFRB fusion gene-positive and BCR-ABL-negative chronic myeloproliferative disorders</title><author><name sortKey="David, Marianna" sort="David, Marianna" uniqKey="David M" first="Marianna" last="David">Marianna David</name><affiliation wicri:level="1"><inist:fA14 i1="01"><s1>Department of Haematology, University of Pecs</s1><s2>Pecs</s2><s3>HUN</s3><sZ>1 aut.</sZ></inist:fA14><country>Hongrie</country><wicri:noRegion>Pecs</wicri:noRegion></affiliation></author><author><name sortKey="Cross, Nicholas C P" sort="Cross, Nicholas C P" uniqKey="Cross N" first="Nicholas C. P." last="Cross">Nicholas C. P. Cross</name><affiliation wicri:level="1"><inist:fA14 i1="02"><s1>Wessex Regional Genetics Laboratory, Salisbury District Hospital</s1><s2>Salisbury</s2><s3>GBR</s3><sZ>2 aut.</sZ><sZ>4 aut.</sZ><sZ>5 aut.</sZ><sZ>9 aut.</sZ><sZ>22 aut.</sZ></inist:fA14><country>Royaume-Uni</country><wicri:noRegion>Salisbury</wicri:noRegion></affiliation></author><author><name sortKey="Burgstaller, Sonja" sort="Burgstaller, Sonja" uniqKey="Burgstaller S" first="Sonja" last="Burgstaller">Sonja Burgstaller</name><affiliation wicri:level="1"><inist:fA14 i1="03"><s1>Department of Internal Medicine, General Hospital</s1><s2>Wels</s2><s3>AUT</s3><sZ>3 aut.</sZ></inist:fA14><country>Autriche</country><wicri:noRegion>Wels</wicri:noRegion></affiliation></author><author><name sortKey="Chase, Andrew" sort="Chase, Andrew" uniqKey="Chase A" first="Andrew" last="Chase">Andrew Chase</name><affiliation wicri:level="1"><inist:fA14 i1="02"><s1>Wessex Regional Genetics Laboratory, Salisbury District Hospital</s1><s2>Salisbury</s2><s3>GBR</s3><sZ>2 aut.</sZ><sZ>4 aut.</sZ><sZ>5 aut.</sZ><sZ>9 aut.</sZ><sZ>22 aut.</sZ></inist:fA14><country>Royaume-Uni</country><wicri:noRegion>Salisbury</wicri:noRegion></affiliation></author><author><name sortKey="Curtis, Claire" sort="Curtis, Claire" uniqKey="Curtis C" first="Claire" last="Curtis">Claire Curtis</name><affiliation wicri:level="1"><inist:fA14 i1="02"><s1>Wessex Regional Genetics Laboratory, Salisbury District Hospital</s1><s2>Salisbury</s2><s3>GBR</s3><sZ>2 aut.</sZ><sZ>4 aut.</sZ><sZ>5 aut.</sZ><sZ>9 aut.</sZ><sZ>22 aut.</sZ></inist:fA14><country>Royaume-Uni</country><wicri:noRegion>Salisbury</wicri:noRegion></affiliation></author><author><name sortKey="Dang, Raymond" sort="Dang, Raymond" uniqKey="Dang R" first="Raymond" last="Dang">Raymond Dang</name><affiliation wicri:level="1"><inist:fA14 i1="04"><s1>Department of Haematology, Dumfries General Infirmary</s1><s2>Dumfries, Scotland</s2><s3>GBR</s3><sZ>6 aut.</sZ><sZ>21 aut.</sZ></inist:fA14><country>Royaume-Uni</country><wicri:noRegion>Dumfries, Scotland</wicri:noRegion></affiliation></author><author><name sortKey="Gardembas, Martine" sort="Gardembas, Martine" uniqKey="Gardembas M" first="Martine" last="Gardembas">Martine Gardembas</name><affiliation wicri:level="3"><inist:fA14 i1="05"><s1>Department of Haematology, Centre Hospitalier Universitaire (CHU) Angers</s1><s2>Angers</s2><s3>FRA</s3><sZ>7 aut.</sZ></inist:fA14><country>France</country><placeName><region type="region">Pays de la Loire</region><region type="old region">Pays de la Loire</region><settlement type="city">Angers</settlement></placeName></affiliation></author><author><name sortKey="Goldman, John M" sort="Goldman, John M" uniqKey="Goldman J" first="John M." last="Goldman">John M. Goldman</name><affiliation wicri:level="3"><inist:fA14 i1="06"><s1>Department of Haematology, Faculty of Medicine, Imperial College</s1><s2>London</s2><s3>GBR</s3><sZ>8 aut.</sZ><sZ>16 aut.</sZ><sZ>24 aut.</sZ></inist:fA14><country>Royaume-Uni</country><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="Grand, Francis" sort="Grand, Francis" uniqKey="Grand F" first="Francis" last="Grand">Francis Grand</name><affiliation wicri:level="1"><inist:fA14 i1="02"><s1>Wessex Regional Genetics Laboratory, Salisbury District Hospital</s1><s2>Salisbury</s2><s3>GBR</s3><sZ>2 aut.</sZ><sZ>4 aut.</sZ><sZ>5 aut.</sZ><sZ>9 aut.</sZ><sZ>22 aut.</sZ></inist:fA14><country>Royaume-Uni</country><wicri:noRegion>Salisbury</wicri:noRegion></affiliation></author><author><name sortKey="Hughes, George" sort="Hughes, George" uniqKey="Hughes G" first="George" last="Hughes">George Hughes</name><affiliation wicri:level="3"><inist:fA14 i1="07"><s1>Department of Haematology, West Middlesex Hospital</s1><s2>London</s2><s3>GBR</s3><sZ>10 aut.</sZ></inist:fA14><country>Royaume-Uni</country><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="Huguet, Francoise" sort="Huguet, Francoise" uniqKey="Huguet F" first="Francoise" last="Huguet">Francoise Huguet</name><affiliation wicri:level="3"><inist:fA14 i1="08"><s1>Department of Haematology, Hopital de Purpan</s1><s2>Toulouse</s2><s3>FRA</s3><sZ>11 aut.</sZ></inist:fA14><country>France</country><placeName><region type="region">Occitanie (région administrative)</region><region type="old region">Midi-Pyrénées</region><settlement type="city">Toulouse</settlement></placeName></affiliation></author><author><name sortKey="Lavender, Louise" sort="Lavender, Louise" uniqKey="Lavender L" first="Louise" last="Lavender">Louise Lavender</name><affiliation wicri:level="1"><inist:fA14 i1="09"><s1>Molecular Pathology Unit, Southampton General Hospital</s1><s2>Southampton</s2><s3>GBR</s3><sZ>12 aut.</sZ></inist:fA14><country>Royaume-Uni</country><wicri:noRegion>Southampton</wicri:noRegion></affiliation></author><author><name sortKey="Mcarthur, Grant A" sort="Mcarthur, Grant A" uniqKey="Mcarthur G" first="Grant A." last="Mcarthur">Grant A. Mcarthur</name><affiliation wicri:level="3"><inist:fA14 i1="10"><s1>Department of Haematology, Peter MacCallum Cancer Centre</s1><s2>Melbourne</s2><s3>AUS</s3><sZ>13 aut.</sZ><sZ>19 aut.</sZ></inist:fA14><country>Australie</country><placeName><settlement type="city">Melbourne</settlement><region type="état">Victoria (État)</region></placeName></affiliation></author><author><name sortKey="Mahon, Francois X" sort="Mahon, Francois X" uniqKey="Mahon F" first="Francois X." last="Mahon">Francois X. Mahon</name><affiliation wicri:level="3"><inist:fA14 i1="11"><s1>Laboratory of normal and pathological haematology, University Victor Ségalen</s1><s2>Bordeaux</s2><s3>FRA</s3><sZ>14 aut.</sZ></inist:fA14><country>France</country><placeName><region type="region">Nouvelle-Aquitaine</region><region type="old region">Aquitaine</region><settlement type="city">Bordeaux</settlement></placeName></affiliation></author><author><name sortKey="Massimini, Giorgio" sort="Massimini, Giorgio" uniqKey="Massimini G" first="Giorgio" last="Massimini">Giorgio Massimini</name><affiliation wicri:level="1"><inist:fA14 i1="12"><s1>Novartis Oncology</s1><s2>Basel</s2><s3>CHE</s3><sZ>15 aut.</sZ><sZ>23 aut.</sZ></inist:fA14><country>Suisse</country><wicri:noRegion>Novartis Oncology</wicri:noRegion></affiliation></author><author><name sortKey="Melo, Junia" sort="Melo, Junia" uniqKey="Melo J" first="Junia" last="Melo">Junia Melo</name><affiliation wicri:level="3"><inist:fA14 i1="06"><s1>Department of Haematology, Faculty of Medicine, Imperial College</s1><s2>London</s2><s3>GBR</s3><sZ>8 aut.</sZ><sZ>16 aut.</sZ><sZ>24 aut.</sZ></inist:fA14><country>Royaume-Uni</country><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="Rousselot, Philippe" sort="Rousselot, Philippe" uniqKey="Rousselot P" first="Philippe" last="Rousselot">Philippe Rousselot</name><affiliation wicri:level="3"><inist:fA14 i1="13"><s1>Department of Haematology, Hopital St Louis</s1><s2>Paris</s2><s3>FRA</s3><sZ>17 aut.</sZ></inist:fA14><country>France</country><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="Russell Jones, Robin J" sort="Russell Jones, Robin J" uniqKey="Russell Jones R" first="Robin J." last="Russell-Jones">Robin J. Russell-Jones</name><affiliation wicri:level="3"><inist:fA14 i1="14"><s1>Skin Tumour Unit, St John's Institute of Dermatology, St Thomas' Hospital, Lambeth Palace Road</s1><s2>London</s2><s3>GBR</s3><sZ>18 aut.</sZ></inist:fA14><country>Royaume-Uni</country><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="Seymour, John F" sort="Seymour, John F" uniqKey="Seymour J" first="John F." last="Seymour">John F. Seymour</name><affiliation wicri:level="3"><inist:fA14 i1="10"><s1>Department of Haematology, Peter MacCallum Cancer Centre</s1><s2>Melbourne</s2><s3>AUS</s3><sZ>13 aut.</sZ><sZ>19 aut.</sZ></inist:fA14><country>Australie</country><placeName><settlement type="city">Melbourne</settlement><region type="état">Victoria (État)</region></placeName></affiliation></author><author><name sortKey="Smith, Graeme" sort="Smith, Graeme" uniqKey="Smith G" first="Graeme" last="Smith">Graeme Smith</name><affiliation wicri:level="1"><inist:fA14 i1="15"><s1>Department of Haematology, Leeds General Infirmary</s1><s2>Leeds</s2><s3>GBR</s3><sZ>20 aut.</sZ></inist:fA14><country>Royaume-Uni</country><wicri:noRegion>Leeds</wicri:noRegion></affiliation></author><author><name sortKey="Stark, Alastair" sort="Stark, Alastair" uniqKey="Stark A" first="Alastair" last="Stark">Alastair Stark</name><affiliation wicri:level="1"><inist:fA14 i1="04"><s1>Department of Haematology, Dumfries General Infirmary</s1><s2>Dumfries, Scotland</s2><s3>GBR</s3><sZ>6 aut.</sZ><sZ>21 aut.</sZ></inist:fA14><country>Royaume-Uni</country><wicri:noRegion>Dumfries, Scotland</wicri:noRegion></affiliation></author><author><name sortKey="Waghorn, Katherine" sort="Waghorn, Katherine" uniqKey="Waghorn K" first="Katherine" last="Waghorn">Katherine Waghorn</name><affiliation wicri:level="1"><inist:fA14 i1="02"><s1>Wessex Regional Genetics Laboratory, Salisbury District Hospital</s1><s2>Salisbury</s2><s3>GBR</s3><sZ>2 aut.</sZ><sZ>4 aut.</sZ><sZ>5 aut.</sZ><sZ>9 aut.</sZ><sZ>22 aut.</sZ></inist:fA14><country>Royaume-Uni</country><wicri:noRegion>Salisbury</wicri:noRegion></affiliation></author><author><name sortKey="Nikolova, Zariana" sort="Nikolova, Zariana" uniqKey="Nikolova Z" first="Zariana" last="Nikolova">Zariana Nikolova</name><affiliation wicri:level="1"><inist:fA14 i1="12"><s1>Novartis Oncology</s1><s2>Basel</s2><s3>CHE</s3><sZ>15 aut.</sZ><sZ>23 aut.</sZ></inist:fA14><country>Suisse</country><wicri:noRegion>Novartis Oncology</wicri:noRegion></affiliation></author><author><name sortKey="Apperley, Jane F" sort="Apperley, Jane F" uniqKey="Apperley J" first="Jane F." last="Apperley">Jane F. Apperley</name><affiliation wicri:level="3"><inist:fA14 i1="06"><s1>Department of Haematology, Faculty of Medicine, Imperial College</s1><s2>London</s2><s3>GBR</s3><sZ>8 aut.</sZ><sZ>16 aut.</sZ><sZ>24 aut.</sZ></inist:fA14><country>Royaume-Uni</country><placeName><settlement type="city">Londres</settlement><region type="country">Angleterre</region><region type="région" nuts="1">Grand Londres</region></placeName></affiliation></author></analytic><series><title level="j" type="main">Blood</title><title level="j" type="abbreviated">Blood</title><idno type="ISSN">0006-4971</idno><imprint><date when="2007">2007</date></imprint></series></biblStruct></sourceDesc><seriesStmt><title level="j" type="main">Blood</title><title level="j" type="abbreviated">Blood</title><idno type="ISSN">0006-4971</idno></seriesStmt></fileDesc><profileDesc><textClass><keywords scheme="KwdEn" xml:lang="en"><term>Abnormal chromosome C9</term><term>Abnormal chromosome G22</term><term>Antineoplastic agent</term><term>Bcr-abl protein</term><term>Chromosome translocation</term><term>Chronic</term><term>Enzyme inhibitor</term><term>Hematology</term><term>Human</term><term>Hybrid gene</term><term>Imatinib</term><term>Myeloproliferative syndrome</term><term>Philadelphia chromosome</term><term>Platelet derived growth factor receptor beta</term><term>Protein kinase</term><term>Protein-tyrosine kinase</term></keywords><keywords scheme="Pascal" xml:lang="fr"><term>Imatinib</term><term>Protein-tyrosine kinase</term><term>Inhibiteur enzyme</term><term>Myéloprolifératif syndrome</term><term>Homme</term><term>Gène hybride</term><term>Translocation chromosomique</term><term>Chromosome C9 anormal</term><term>Chromosome G22 anormal</term><term>Chromosome Ph1</term><term>Protein kinase</term><term>Chronique</term><term>Hématologie</term><term>Anticancéreux</term><term>Gène bcr</term><term>Gène abl</term><term>Récepteur PDGFRB</term><term>Protéine bcr-abl</term></keywords><keywords scheme="Wicri" type="topic" xml:lang="fr"><term>Homme</term></keywords></textClass></profileDesc></teiHeader><front><div type="abstract" xml:lang="en">Fusion genes derived from the platelet-derived growth factor receptor beta (PDGFRB) or alpha (PDGFRA) play an important role in the pathogenesis of BCR-ABL-negative chronic myeloproliferative disorders (CMPDs). These fusion genes encode constitutively activated receptor tyrosine kinases that can be inhibited by imatinib. Twelve patients with BCR-ABL-negative CMPDs and reciprocal translocations involving PDGFRB received imatinib for a median of 47 months (range, 0.1-60 months). Eleven had prompt responses with normalization of peripheral-blood cell counts and disappearance of eosinophilia; 10 had complete resolution of cytogenetic abnormalities and decrease or disappearance of fusion transcripts as measured by reverse transcriptase-polymerase chain reaction (RT-PCR). Updates were sought from 8 further patients previously described in the literature; prompt responses were described in 7 and persist in 6. Our data show that durable hematologic and cytogenetic responses are achieved with imatinib in patients with PDGFRB fusion-positive, BCR-ABL-negative CMPDs.</div></front></TEI><affiliations><list><country><li>Australie</li><li>Autriche</li><li>France</li><li>Hongrie</li><li>Royaume-Uni</li><li>Suisse</li></country><region><li>Angleterre</li><li>Aquitaine</li><li>Grand Londres</li><li>Midi-Pyrénées</li><li>Nouvelle-Aquitaine</li><li>Occitanie (région administrative)</li><li>Pays de la Loire</li><li>Victoria (État)</li><li>Île-de-France</li></region><settlement><li>Angers</li><li>Bordeaux</li><li>Londres</li><li>Melbourne</li><li>Paris</li><li>Toulouse</li></settlement></list><tree><country name="Hongrie"><noRegion><name sortKey="David, Marianna" sort="David, Marianna" uniqKey="David M" first="Marianna" last="David">Marianna David</name></noRegion></country><country name="Royaume-Uni"><noRegion><name sortKey="Cross, Nicholas C P" sort="Cross, Nicholas C P" uniqKey="Cross N" first="Nicholas C. P." last="Cross">Nicholas C. P. Cross</name></noRegion><name sortKey="Apperley, Jane F" sort="Apperley, Jane F" uniqKey="Apperley J" first="Jane F." last="Apperley">Jane F. Apperley</name><name sortKey="Chase, Andrew" sort="Chase, Andrew" uniqKey="Chase A" first="Andrew" last="Chase">Andrew Chase</name><name sortKey="Curtis, Claire" sort="Curtis, Claire" uniqKey="Curtis C" first="Claire" last="Curtis">Claire Curtis</name><name sortKey="Dang, Raymond" sort="Dang, Raymond" uniqKey="Dang R" first="Raymond" last="Dang">Raymond Dang</name><name sortKey="Goldman, John M" sort="Goldman, John M" uniqKey="Goldman J" first="John M." last="Goldman">John M. Goldman</name><name sortKey="Grand, Francis" sort="Grand, Francis" uniqKey="Grand F" first="Francis" last="Grand">Francis Grand</name><name sortKey="Hughes, George" sort="Hughes, George" uniqKey="Hughes G" first="George" last="Hughes">George Hughes</name><name sortKey="Lavender, Louise" sort="Lavender, Louise" uniqKey="Lavender L" first="Louise" last="Lavender">Louise Lavender</name><name sortKey="Melo, Junia" sort="Melo, Junia" uniqKey="Melo J" first="Junia" last="Melo">Junia Melo</name><name sortKey="Russell Jones, Robin J" sort="Russell Jones, Robin J" uniqKey="Russell Jones R" first="Robin J." last="Russell-Jones">Robin J. Russell-Jones</name><name sortKey="Smith, Graeme" sort="Smith, Graeme" uniqKey="Smith G" first="Graeme" last="Smith">Graeme Smith</name><name sortKey="Stark, Alastair" sort="Stark, Alastair" uniqKey="Stark A" first="Alastair" last="Stark">Alastair Stark</name><name sortKey="Waghorn, Katherine" sort="Waghorn, Katherine" uniqKey="Waghorn K" first="Katherine" last="Waghorn">Katherine Waghorn</name></country><country name="Autriche"><noRegion><name sortKey="Burgstaller, Sonja" sort="Burgstaller, Sonja" uniqKey="Burgstaller S" first="Sonja" last="Burgstaller">Sonja Burgstaller</name></noRegion></country><country name="France"><region name="Pays de la Loire"><name sortKey="Gardembas, Martine" sort="Gardembas, Martine" uniqKey="Gardembas M" first="Martine" last="Gardembas">Martine Gardembas</name></region><name sortKey="Huguet, Francoise" sort="Huguet, Francoise" uniqKey="Huguet F" first="Francoise" last="Huguet">Francoise Huguet</name><name sortKey="Mahon, Francois X" sort="Mahon, Francois X" uniqKey="Mahon F" first="Francois X." last="Mahon">Francois X. Mahon</name><name sortKey="Rousselot, Philippe" sort="Rousselot, Philippe" uniqKey="Rousselot P" first="Philippe" last="Rousselot">Philippe Rousselot</name></country><country name="Australie"><region name="Victoria (État)"><name sortKey="Mcarthur, Grant A" sort="Mcarthur, Grant A" uniqKey="Mcarthur G" first="Grant A." last="Mcarthur">Grant A. Mcarthur</name></region><name sortKey="Seymour, John F" sort="Seymour, John F" uniqKey="Seymour J" first="John F." last="Seymour">John F. Seymour</name></country><country name="Suisse"><noRegion><name sortKey="Massimini, Giorgio" sort="Massimini, Giorgio" uniqKey="Massimini G" first="Giorgio" last="Massimini">Giorgio Massimini</name></noRegion><name sortKey="Nikolova, Zariana" sort="Nikolova, Zariana" uniqKey="Nikolova Z" first="Zariana" last="Nikolova">Zariana Nikolova</name></country></tree></affiliations></record>Pour manipuler ce document sous Unix (Dilib)
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