Pretreatment characteristics and clinical outcome of acute promyelocytic leukaemia patients according to the PML‐RARα isoforms: a study of the PETHEMA group

Of 167 newly diagnosed acute promyelocytic leukaemia patients, 83 patients were long (L)‐form (50%), eight variable (V)‐form (5%) and 76 short (S)‐form (45%). The V‐form and S‐form groups presented a significantly higher percentage of patients with white blood cell counts > 10 × 109/l (P ...

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Veröffentlicht in:British journal of haematology 2001-07, Vol.114 (1), p.99-103
Hauptverfasser: González, M., Barragán, E., Bolufer, P., Chillón, C., Colomer, D., Borstein, R., Calasanz, M. J., Gómez‐Casares, M. T., Villegas, A., Marugán, I., Román, J., Martín, G., Rayón, C., Debén, G., Tormo, M., Díaz‐Mediavilla, J., Esteve, J., González‐San Miguel, J., Rivas, C., Pérez‐Equiza, K., García‐Sanz, R., Capote, F. J., Ribera, J. M., Arias, J., León, A., Sanz, M. A.
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container_issue 1
container_start_page 99
container_title British journal of haematology
container_volume 114
creator González, M.
Barragán, E.
Bolufer, P.
Chillón, C.
Colomer, D.
Borstein, R.
Calasanz, M. J.
Gómez‐Casares, M. T.
Villegas, A.
Marugán, I.
Román, J.
Martín, G.
Rayón, C.
Debén, G.
Tormo, M.
Díaz‐Mediavilla, J.
Esteve, J.
González‐San Miguel, J.
Rivas, C.
Pérez‐Equiza, K.
García‐Sanz, R.
Capote, F. J.
Ribera, J. M.
Arias, J.
León, A.
Sanz, M. A.
description Of 167 newly diagnosed acute promyelocytic leukaemia patients, 83 patients were long (L)‐form (50%), eight variable (V)‐form (5%) and 76 short (S)‐form (45%). The V‐form and S‐form groups presented a significantly higher percentage of patients with white blood cell counts > 10 × 109/l (P 
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J. ; Gómez‐Casares, M. T. ; Villegas, A. ; Marugán, I. ; Román, J. ; Martín, G. ; Rayón, C. ; Debén, G. ; Tormo, M. ; Díaz‐Mediavilla, J. ; Esteve, J. ; González‐San Miguel, J. ; Rivas, C. ; Pérez‐Equiza, K. ; García‐Sanz, R. ; Capote, F. J. ; Ribera, J. M. ; Arias, J. ; León, A. ; Sanz, M. A.</creator><creatorcontrib>González, M. ; Barragán, E. ; Bolufer, P. ; Chillón, C. ; Colomer, D. ; Borstein, R. ; Calasanz, M. J. ; Gómez‐Casares, M. T. ; Villegas, A. ; Marugán, I. ; Román, J. ; Martín, G. ; Rayón, C. ; Debén, G. ; Tormo, M. ; Díaz‐Mediavilla, J. ; Esteve, J. ; González‐San Miguel, J. ; Rivas, C. ; Pérez‐Equiza, K. ; García‐Sanz, R. ; Capote, F. J. ; Ribera, J. M. ; Arias, J. ; León, A. ; Sanz, M. A. ; Spanish Programme for the Study and Treatment of Haematological Malignancies (PETHEMA) Group ; for the Spanish Programme for the Study and Treatment of Haematological Malignancies (PETHEMA) Group</creatorcontrib><description>Of 167 newly diagnosed acute promyelocytic leukaemia patients, 83 patients were long (L)‐form (50%), eight variable (V)‐form (5%) and 76 short (S)‐form (45%). The V‐form and S‐form groups presented a significantly higher percentage of patients with white blood cell counts &gt; 10 × 109/l (P &lt; 0·05). The S‐form cases displayed a significantly higher number of cases with M3v microgranular features (P = 0·005) and CD34 expression (P &lt; 0·0001). There were no differences between the three isoforms in complete remission (CR) rate (overall CR 90%), but the 3‐year disease‐free survival was lower for V‐form cases than it was for L‐ and S‐form cases (62% vs. 94% and 89%, P = 0·056). We conclude that the V‐form and S‐form types are associated with some negative prognostic features at diagnosis. However, our data were only able to demonstrate an association with adverse prognosis in the V‐form type and, moreover, as the number of cases was limited, needs to be confirmed in large, uniformly treated series.</description><identifier>ISSN: 0007-1048</identifier><identifier>EISSN: 1365-2141</identifier><identifier>DOI: 10.1046/j.1365-2141.2001.02915.x</identifier><identifier>PMID: 11472351</identifier><identifier>CODEN: BJHEAL</identifier><language>eng</language><publisher>Oxford, UK: Blackwell Science Ltd</publisher><subject>acute promyelocytic leukaemia ; Adolescent ; Adult ; Aged ; all‐trans retinoic acid (ATRA) ; Antigens, CD34 - analysis ; Antineoplastic Combined Chemotherapy Protocols - therapeutic use ; Biological and medical sciences ; Child ; Child, Preschool ; clinical outcome ; complete remission ; Disease-Free Survival ; Female ; Hematologic and hematopoietic diseases ; Humans ; Infant ; Infant, Newborn ; Leukemia, Promyelocytic, Acute - drug therapy ; Leukemia, Promyelocytic, Acute - immunology ; Leukemia, Promyelocytic, Acute - metabolism ; Leukemias. Malignant lymphomas. Malignant reticulosis. Myelofibrosis ; Leukocyte Count ; Male ; Medical sciences ; Middle Aged ; Neoplasm Proteins - genetics ; Oncogene Proteins, Fusion - genetics ; PML/RARα isoform ; Polymerase Chain Reaction - methods ; Prognosis ; Proportional Hazards Models ; Protein Isoforms - genetics ; reverse transcription–polymerase chain reaction (RT–PCR) ; Treatment Outcome ; Tretinoin - therapeutic use</subject><ispartof>British journal of haematology, 2001-07, Vol.114 (1), p.99-103</ispartof><rights>2002 INIST-CNRS</rights><lds50>peer_reviewed</lds50><oa>free_for_read</oa><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c4455-615b16cbc55175130cb656399204e5344a956555f8952543aa644f0fb094c7193</citedby><cites>FETCH-LOGICAL-c4455-615b16cbc55175130cb656399204e5344a956555f8952543aa644f0fb094c7193</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktopdf>$$Uhttps://onlinelibrary.wiley.com/doi/pdf/10.1046%2Fj.1365-2141.2001.02915.x$$EPDF$$P50$$Gwiley$$H</linktopdf><linktohtml>$$Uhttps://onlinelibrary.wiley.com/doi/full/10.1046%2Fj.1365-2141.2001.02915.x$$EHTML$$P50$$Gwiley$$H</linktohtml><link.rule.ids>314,776,780,1411,1427,27901,27902,45550,45551,46384,46808</link.rule.ids><backlink>$$Uhttp://pascal-francis.inist.fr/vibad/index.php?action=getRecordDetail&amp;idt=14065976$$DView record in Pascal Francis$$Hfree_for_read</backlink><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/11472351$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>González, M.</creatorcontrib><creatorcontrib>Barragán, E.</creatorcontrib><creatorcontrib>Bolufer, P.</creatorcontrib><creatorcontrib>Chillón, C.</creatorcontrib><creatorcontrib>Colomer, D.</creatorcontrib><creatorcontrib>Borstein, R.</creatorcontrib><creatorcontrib>Calasanz, M. J.</creatorcontrib><creatorcontrib>Gómez‐Casares, M. T.</creatorcontrib><creatorcontrib>Villegas, A.</creatorcontrib><creatorcontrib>Marugán, I.</creatorcontrib><creatorcontrib>Román, J.</creatorcontrib><creatorcontrib>Martín, G.</creatorcontrib><creatorcontrib>Rayón, C.</creatorcontrib><creatorcontrib>Debén, G.</creatorcontrib><creatorcontrib>Tormo, M.</creatorcontrib><creatorcontrib>Díaz‐Mediavilla, J.</creatorcontrib><creatorcontrib>Esteve, J.</creatorcontrib><creatorcontrib>González‐San Miguel, J.</creatorcontrib><creatorcontrib>Rivas, C.</creatorcontrib><creatorcontrib>Pérez‐Equiza, K.</creatorcontrib><creatorcontrib>García‐Sanz, R.</creatorcontrib><creatorcontrib>Capote, F. J.</creatorcontrib><creatorcontrib>Ribera, J. M.</creatorcontrib><creatorcontrib>Arias, J.</creatorcontrib><creatorcontrib>León, A.</creatorcontrib><creatorcontrib>Sanz, M. A.</creatorcontrib><creatorcontrib>Spanish Programme for the Study and Treatment of Haematological Malignancies (PETHEMA) Group</creatorcontrib><creatorcontrib>for the Spanish Programme for the Study and Treatment of Haematological Malignancies (PETHEMA) Group</creatorcontrib><title>Pretreatment characteristics and clinical outcome of acute promyelocytic leukaemia patients according to the PML‐RARα isoforms: a study of the PETHEMA group</title><title>British journal of haematology</title><addtitle>Br J Haematol</addtitle><description>Of 167 newly diagnosed acute promyelocytic leukaemia patients, 83 patients were long (L)‐form (50%), eight variable (V)‐form (5%) and 76 short (S)‐form (45%). The V‐form and S‐form groups presented a significantly higher percentage of patients with white blood cell counts &gt; 10 × 109/l (P &lt; 0·05). The S‐form cases displayed a significantly higher number of cases with M3v microgranular features (P = 0·005) and CD34 expression (P &lt; 0·0001). There were no differences between the three isoforms in complete remission (CR) rate (overall CR 90%), but the 3‐year disease‐free survival was lower for V‐form cases than it was for L‐ and S‐form cases (62% vs. 94% and 89%, P = 0·056). We conclude that the V‐form and S‐form types are associated with some negative prognostic features at diagnosis. However, our data were only able to demonstrate an association with adverse prognosis in the V‐form type and, moreover, as the number of cases was limited, needs to be confirmed in large, uniformly treated series.</description><subject>acute promyelocytic leukaemia</subject><subject>Adolescent</subject><subject>Adult</subject><subject>Aged</subject><subject>all‐trans retinoic acid (ATRA)</subject><subject>Antigens, CD34 - analysis</subject><subject>Antineoplastic Combined Chemotherapy Protocols - therapeutic use</subject><subject>Biological and medical sciences</subject><subject>Child</subject><subject>Child, Preschool</subject><subject>clinical outcome</subject><subject>complete remission</subject><subject>Disease-Free Survival</subject><subject>Female</subject><subject>Hematologic and hematopoietic diseases</subject><subject>Humans</subject><subject>Infant</subject><subject>Infant, Newborn</subject><subject>Leukemia, Promyelocytic, Acute - drug therapy</subject><subject>Leukemia, Promyelocytic, Acute - immunology</subject><subject>Leukemia, Promyelocytic, Acute - metabolism</subject><subject>Leukemias. Malignant lymphomas. Malignant reticulosis. Myelofibrosis</subject><subject>Leukocyte Count</subject><subject>Male</subject><subject>Medical sciences</subject><subject>Middle Aged</subject><subject>Neoplasm Proteins - genetics</subject><subject>Oncogene Proteins, Fusion - genetics</subject><subject>PML/RARα isoform</subject><subject>Polymerase Chain Reaction - methods</subject><subject>Prognosis</subject><subject>Proportional Hazards Models</subject><subject>Protein Isoforms - genetics</subject><subject>reverse transcription–polymerase chain reaction (RT–PCR)</subject><subject>Treatment Outcome</subject><subject>Tretinoin - therapeutic use</subject><issn>0007-1048</issn><issn>1365-2141</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2001</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNqNkUFu1DAUhi0EokPLFZA3sEuwEz9njMRiqAaGaiqqqqwtx-O0HpJ4sB3R7DgCN-AMXIRDcJI6nRHddmVL_v7ffv4QwpTklDD-dpvTkkNWUEbzghCak0JQyG-foNn_g6doRgipshSYH6EXIWwTWBKgz9ERpawqSqAz9PvCm-iNip3pI9Y3yisdjbchWh2w6jdYt7a3WrXYDVG7zmDXYKWHaPDOu240rdNjgnFrhm_KdFbhnYo2taW41s5vbH-No8PxxuCL8_W_n78uF5d__2AbXON8F95hhUMcNuNUfA8tr1bL8wW-9m7YnaBnjWqDeXlYj9HXj8ur01W2_vLp8-linWnGADJOoaZc1xqAVpDG1DUHXgpREGagZEwJ4ADQzAUUwEqlOGMNaWoimK6oKI_Rm31vGur7YEKUnQ3atK3qjRuCrCgpuahoAud7UHsXgjeN3HnbKT9KSuQkR27l5EBODuQkR97Lkbcp-upwx1B3ZvMQPNhIwOsDoEL68carXtvwwDHCQVQ8ce_33A_bmvHRD5AfzlbTrrwDSmysnw</recordid><startdate>200107</startdate><enddate>200107</enddate><creator>González, M.</creator><creator>Barragán, E.</creator><creator>Bolufer, P.</creator><creator>Chillón, C.</creator><creator>Colomer, D.</creator><creator>Borstein, R.</creator><creator>Calasanz, M. 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The V‐form and S‐form groups presented a significantly higher percentage of patients with white blood cell counts &gt; 10 × 109/l (P &lt; 0·05). The S‐form cases displayed a significantly higher number of cases with M3v microgranular features (P = 0·005) and CD34 expression (P &lt; 0·0001). There were no differences between the three isoforms in complete remission (CR) rate (overall CR 90%), but the 3‐year disease‐free survival was lower for V‐form cases than it was for L‐ and S‐form cases (62% vs. 94% and 89%, P = 0·056). We conclude that the V‐form and S‐form types are associated with some negative prognostic features at diagnosis. 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issn 0007-1048
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source MEDLINE; Wiley Online Library; Free E-Journal (出版社公開部分のみ)
subjects acute promyelocytic leukaemia
Adolescent
Adult
Aged
all‐trans retinoic acid (ATRA)
Antigens, CD34 - analysis
Antineoplastic Combined Chemotherapy Protocols - therapeutic use
Biological and medical sciences
Child
Child, Preschool
clinical outcome
complete remission
Disease-Free Survival
Female
Hematologic and hematopoietic diseases
Humans
Infant
Infant, Newborn
Leukemia, Promyelocytic, Acute - drug therapy
Leukemia, Promyelocytic, Acute - immunology
Leukemia, Promyelocytic, Acute - metabolism
Leukemias. Malignant lymphomas. Malignant reticulosis. Myelofibrosis
Leukocyte Count
Male
Medical sciences
Middle Aged
Neoplasm Proteins - genetics
Oncogene Proteins, Fusion - genetics
PML/RARα isoform
Polymerase Chain Reaction - methods
Prognosis
Proportional Hazards Models
Protein Isoforms - genetics
reverse transcription–polymerase chain reaction (RT–PCR)
Treatment Outcome
Tretinoin - therapeutic use
title Pretreatment characteristics and clinical outcome of acute promyelocytic leukaemia patients according to the PML‐RARα isoforms: a study of the PETHEMA group
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