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<title xml:lang="en">Outcome and pathologic classification of children and adolescents with mediastinal large B-cell lymphoma treated with FAB/LMB96 mature B-NHL therapy</title>
<author>
<name sortKey="Gerrard, Mary" sort="Gerrard, Mary" uniqKey="Gerrard M" first="Mary" last="Gerrard">Mary Gerrard</name>
<affiliation>
<nlm:aff id="aff1">Sheffield Children's Hospital, Sheffield, United Kingdom;</nlm:aff>
</affiliation>
</author>
<author>
<name sortKey="Waxman, Ian M" sort="Waxman, Ian M" uniqKey="Waxman I" first="Ian M." last="Waxman">Ian M. Waxman</name>
<affiliation>
<nlm:aff id="aff2">Columbia University, New York, NY;</nlm:aff>
</affiliation>
</author>
<author>
<name sortKey="Sposto, Richard" sort="Sposto, Richard" uniqKey="Sposto R" first="Richard" last="Sposto">Richard Sposto</name>
<affiliation>
<nlm:aff id="aff3">Keck School of Medicine, University of Southern California, Los Angeles, CA;</nlm:aff>
</affiliation>
</author>
<author>
<name sortKey="Auperin, Anne" sort="Auperin, Anne" uniqKey="Auperin A" first="Anne" last="Auperin">Anne Auperin</name>
<affiliation>
<nlm:aff id="aff4">Institut Gustave Roussy, Paris, France;</nlm:aff>
</affiliation>
</author>
<author>
<name sortKey="Perkins, Sherrie L" sort="Perkins, Sherrie L" uniqKey="Perkins S" first="Sherrie L." last="Perkins">Sherrie L. Perkins</name>
<affiliation>
<nlm:aff id="aff5">University of Utah Health Sciences Center, Salt Lake City, UT;</nlm:aff>
</affiliation>
</author>
<author>
<name sortKey="Goldman, Stanton" sort="Goldman, Stanton" uniqKey="Goldman S" first="Stanton" last="Goldman">Stanton Goldman</name>
<affiliation>
<nlm:aff id="aff6"> Medical City Children's Hospital, Dallas, TX;</nlm:aff>
</affiliation>
</author>
<author>
<name sortKey="Harrison, Lauren" sort="Harrison, Lauren" uniqKey="Harrison L" first="Lauren" last="Harrison">Lauren Harrison</name>
<affiliation>
<nlm:aff id="aff7">New York Medical College, Valhalla, NY;</nlm:aff>
</affiliation>
</author>
<author>
<name sortKey="Pinkerton, Ross" sort="Pinkerton, Ross" uniqKey="Pinkerton R" first="Ross" last="Pinkerton">Ross Pinkerton</name>
<affiliation>
<nlm:aff id="aff8">University of Queensland, Royal Children's Hospital, Brisbane, Australia;</nlm:aff>
</affiliation>
</author>
<author>
<name sortKey="Mccarthy, Keith" sort="Mccarthy, Keith" uniqKey="Mccarthy K" first="Keith" last="Mccarthy">Keith Mccarthy</name>
<affiliation>
<nlm:aff wicri:cut="; and" id="aff9">Gloucestershire Hospitals, National Health Service Foundation Trust, Gloucestershire, United Kingdom</nlm:aff>
</affiliation>
</author>
<author>
<name sortKey="Raphael, Martine" sort="Raphael, Martine" uniqKey="Raphael M" first="Martine" last="Raphael">Martine Raphael</name>
<affiliation>
<nlm:aff id="aff10">Centre Hospitalier Universitaire Bicetre, Assistance Publique–Hopitaux de Paris, University Paris Sud 11, Paris, France</nlm:aff>
</affiliation>
</author>
<author>
<name sortKey="Patte, Catherine" sort="Patte, Catherine" uniqKey="Patte C" first="Catherine" last="Patte">Catherine Patte</name>
<affiliation>
<nlm:aff id="aff4">Institut Gustave Roussy, Paris, France;</nlm:aff>
</affiliation>
</author>
<author>
<name sortKey="Cairo, Mitchell S" sort="Cairo, Mitchell S" uniqKey="Cairo M" first="Mitchell S." last="Cairo">Mitchell S. Cairo</name>
<affiliation>
<nlm:aff id="aff7">New York Medical College, Valhalla, NY;</nlm:aff>
</affiliation>
</author>
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<idno type="wicri:source">PMC</idno>
<idno type="pmid">23149845</idno>
<idno type="pmc">3544113</idno>
<idno type="url">http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3544113</idno>
<idno type="RBID">PMC:3544113</idno>
<idno type="doi">10.1182/blood-2012-04-422709</idno>
<date when="2013">2013</date>
<idno type="wicri:Area/Pmc/Corpus">001798</idno>
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<title xml:lang="en" level="a" type="main">Outcome and pathologic classification of children and adolescents with mediastinal large B-cell lymphoma treated with FAB/LMB96 mature B-NHL therapy</title>
<author>
<name sortKey="Gerrard, Mary" sort="Gerrard, Mary" uniqKey="Gerrard M" first="Mary" last="Gerrard">Mary Gerrard</name>
<affiliation>
<nlm:aff id="aff1">Sheffield Children's Hospital, Sheffield, United Kingdom;</nlm:aff>
</affiliation>
</author>
<author>
<name sortKey="Waxman, Ian M" sort="Waxman, Ian M" uniqKey="Waxman I" first="Ian M." last="Waxman">Ian M. Waxman</name>
<affiliation>
<nlm:aff id="aff2">Columbia University, New York, NY;</nlm:aff>
</affiliation>
</author>
<author>
<name sortKey="Sposto, Richard" sort="Sposto, Richard" uniqKey="Sposto R" first="Richard" last="Sposto">Richard Sposto</name>
<affiliation>
<nlm:aff id="aff3">Keck School of Medicine, University of Southern California, Los Angeles, CA;</nlm:aff>
</affiliation>
</author>
<author>
<name sortKey="Auperin, Anne" sort="Auperin, Anne" uniqKey="Auperin A" first="Anne" last="Auperin">Anne Auperin</name>
<affiliation>
<nlm:aff id="aff4">Institut Gustave Roussy, Paris, France;</nlm:aff>
</affiliation>
</author>
<author>
<name sortKey="Perkins, Sherrie L" sort="Perkins, Sherrie L" uniqKey="Perkins S" first="Sherrie L." last="Perkins">Sherrie L. Perkins</name>
<affiliation>
<nlm:aff id="aff5">University of Utah Health Sciences Center, Salt Lake City, UT;</nlm:aff>
</affiliation>
</author>
<author>
<name sortKey="Goldman, Stanton" sort="Goldman, Stanton" uniqKey="Goldman S" first="Stanton" last="Goldman">Stanton Goldman</name>
<affiliation>
<nlm:aff id="aff6"> Medical City Children's Hospital, Dallas, TX;</nlm:aff>
</affiliation>
</author>
<author>
<name sortKey="Harrison, Lauren" sort="Harrison, Lauren" uniqKey="Harrison L" first="Lauren" last="Harrison">Lauren Harrison</name>
<affiliation>
<nlm:aff id="aff7">New York Medical College, Valhalla, NY;</nlm:aff>
</affiliation>
</author>
<author>
<name sortKey="Pinkerton, Ross" sort="Pinkerton, Ross" uniqKey="Pinkerton R" first="Ross" last="Pinkerton">Ross Pinkerton</name>
<affiliation>
<nlm:aff id="aff8">University of Queensland, Royal Children's Hospital, Brisbane, Australia;</nlm:aff>
</affiliation>
</author>
<author>
<name sortKey="Mccarthy, Keith" sort="Mccarthy, Keith" uniqKey="Mccarthy K" first="Keith" last="Mccarthy">Keith Mccarthy</name>
<affiliation>
<nlm:aff wicri:cut="; and" id="aff9">Gloucestershire Hospitals, National Health Service Foundation Trust, Gloucestershire, United Kingdom</nlm:aff>
</affiliation>
</author>
<author>
<name sortKey="Raphael, Martine" sort="Raphael, Martine" uniqKey="Raphael M" first="Martine" last="Raphael">Martine Raphael</name>
<affiliation>
<nlm:aff id="aff10">Centre Hospitalier Universitaire Bicetre, Assistance Publique–Hopitaux de Paris, University Paris Sud 11, Paris, France</nlm:aff>
</affiliation>
</author>
<author>
<name sortKey="Patte, Catherine" sort="Patte, Catherine" uniqKey="Patte C" first="Catherine" last="Patte">Catherine Patte</name>
<affiliation>
<nlm:aff id="aff4">Institut Gustave Roussy, Paris, France;</nlm:aff>
</affiliation>
</author>
<author>
<name sortKey="Cairo, Mitchell S" sort="Cairo, Mitchell S" uniqKey="Cairo M" first="Mitchell S." last="Cairo">Mitchell S. Cairo</name>
<affiliation>
<nlm:aff id="aff7">New York Medical College, Valhalla, NY;</nlm:aff>
</affiliation>
</author>
</analytic>
<series>
<title level="j">Blood</title>
<idno type="ISSN">0006-4971</idno>
<idno type="eISSN">1528-0020</idno>
<imprint>
<date when="2013">2013</date>
</imprint>
</series>
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<front>
<div type="abstract" xml:lang="en">
<p>Mediastinal large B-cell lymphoma (MLBL) represents 2% of mature B-cell non-Hodgkin lymphoma in patients ≤ 18 years of age. We analyzed data from childhood and adolescent patients with stage III MLBL (n = 42) and non-MLBL DLBCL (n = 69) treated with Group B therapy in the French-American-British/Lymphome Malins de Burkitt (FAB/LMB) 96 study. MLBL patients had a male/female 26/16; median age, 15.7 years (range, 12.5-19.7); and LDH < 2 versus ≥ 2 × the upper limit of normal, 23:19. Six MLBL patients (14%) had < a 20% response to initial COP (cyclophosphamide, vincristine, and prednisone) therapy. Central pathology revealed approximately 50% with classical features of primary MLBL. Five-year event-free survival for the stage III MLBL and non-MLBL DLBCL groups was 66% (95% confidence interval [CI], 49%-78%) and 85% (95% CI, 71%-92%), respectively (
<italic>P</italic>
< .001; 14%). The 5-year overall survival in the 42 MLBL patients was 73% (95% CI, 56%-84%). We conclude that MLBL in adolescent patients is associated with significantly inferior event-free survival compared with stage III non-MLBL DLBCL and can be of multiple histologies. Alternate treatment strategies should be investigated in the future taking into account both adult MLBL approaches and more recent biologic findings in adult MLBL.</p>
</div>
</front>
</TEI>
<pmc article-type="research-article">
<pmc-comment>The publisher of this article does not allow downloading of the full text in XML form.</pmc-comment>
<front>
<journal-meta>
<journal-id journal-id-type="nlm-ta">Blood</journal-id>
<journal-id journal-id-type="iso-abbrev">Blood</journal-id>
<journal-id journal-id-type="hwp">bloodjournal</journal-id>
<journal-id journal-id-type="pmc">blood</journal-id>
<journal-id journal-id-type="publisher-id">Blood</journal-id>
<journal-title-group>
<journal-title>Blood</journal-title>
</journal-title-group>
<issn pub-type="ppub">0006-4971</issn>
<issn pub-type="epub">1528-0020</issn>
<publisher>
<publisher-name>American Society of Hematology</publisher-name>
<publisher-loc>Washington, DC</publisher-loc>
</publisher>
</journal-meta>
<article-meta>
<article-id pub-id-type="pmid">23149845</article-id>
<article-id pub-id-type="pmc">3544113</article-id>
<article-id pub-id-type="publisher-id">2012/422709</article-id>
<article-id pub-id-type="doi">10.1182/blood-2012-04-422709</article-id>
<article-categories>
<subj-group subj-group-type="heading">
<subject>Clinical Trials and Observations</subject>
</subj-group>
</article-categories>
<title-group>
<article-title>Outcome and pathologic classification of children and adolescents with mediastinal large B-cell lymphoma treated with FAB/LMB96 mature B-NHL therapy</article-title>
</title-group>
<contrib-group>
<contrib contrib-type="author">
<name>
<surname>Gerrard</surname>
<given-names>Mary</given-names>
</name>
<xref ref-type="aff" rid="aff1">1</xref>
<xref ref-type="author-notes" rid="FN1">*</xref>
</contrib>
<contrib contrib-type="author">
<name>
<surname>Waxman</surname>
<given-names>Ian M.</given-names>
</name>
<xref ref-type="aff" rid="aff2">2</xref>
<xref ref-type="author-notes" rid="FN1">*</xref>
</contrib>
<contrib contrib-type="author">
<name>
<surname>Sposto</surname>
<given-names>Richard</given-names>
</name>
<xref ref-type="aff" rid="aff3">3</xref>
</contrib>
<contrib contrib-type="author">
<name>
<surname>Auperin</surname>
<given-names>Anne</given-names>
</name>
<xref ref-type="aff" rid="aff4">4</xref>
</contrib>
<contrib contrib-type="author">
<name>
<surname>Perkins</surname>
<given-names>Sherrie L.</given-names>
</name>
<xref ref-type="aff" rid="aff5">5</xref>
</contrib>
<contrib contrib-type="author">
<name>
<surname>Goldman</surname>
<given-names>Stanton</given-names>
</name>
<xref ref-type="aff" rid="aff6">6</xref>
</contrib>
<contrib contrib-type="author">
<name>
<surname>Harrison</surname>
<given-names>Lauren</given-names>
</name>
<xref ref-type="aff" rid="aff7">7</xref>
</contrib>
<contrib contrib-type="author">
<name>
<surname>Pinkerton</surname>
<given-names>Ross</given-names>
</name>
<xref ref-type="aff" rid="aff8">8</xref>
</contrib>
<contrib contrib-type="author">
<name>
<surname>McCarthy</surname>
<given-names>Keith</given-names>
</name>
<xref ref-type="aff" rid="aff9">9</xref>
</contrib>
<contrib contrib-type="author">
<name>
<surname>Raphael</surname>
<given-names>Martine</given-names>
</name>
<xref ref-type="aff" rid="aff10">10</xref>
</contrib>
<contrib contrib-type="author">
<name>
<surname>Patte</surname>
<given-names>Catherine</given-names>
</name>
<xref ref-type="aff" rid="aff4">4</xref>
<xref ref-type="author-notes" rid="FN2"></xref>
</contrib>
<contrib contrib-type="author" corresp="yes">
<name>
<surname>Cairo</surname>
<given-names>Mitchell S.</given-names>
</name>
<xref ref-type="aff" rid="aff7">7</xref>
<xref ref-type="author-notes" rid="FN2"></xref>
</contrib>
<on-behalf-of>on behalf of the French-American-British/Lymphome Malins de Burkitt 96 (FAB/LMB 96) International Study Committee</on-behalf-of>
<aff id="aff1">
<label>1</label>
Sheffield Children's Hospital, Sheffield, United Kingdom;</aff>
<aff id="aff2">
<label>2</label>
Columbia University, New York, NY;</aff>
<aff id="aff3">
<label>3</label>
Keck School of Medicine, University of Southern California, Los Angeles, CA;</aff>
<aff id="aff4">
<label>4</label>
Institut Gustave Roussy, Paris, France;</aff>
<aff id="aff5">
<label>5</label>
University of Utah Health Sciences Center, Salt Lake City, UT;</aff>
<aff id="aff6">
<label>6</label>
Medical City Children's Hospital, Dallas, TX;</aff>
<aff id="aff7">
<label>7</label>
New York Medical College, Valhalla, NY;</aff>
<aff id="aff8">
<label>8</label>
University of Queensland, Royal Children's Hospital, Brisbane, Australia;</aff>
<aff id="aff9">
<label>9</label>
Gloucestershire Hospitals, National Health Service Foundation Trust, Gloucestershire, United Kingdom; and</aff>
<aff id="aff10">
<label>10</label>
Centre Hospitalier Universitaire Bicetre, Assistance Publique–Hopitaux de Paris, University Paris Sud 11, Paris, France</aff>
</contrib-group>
<author-notes>
<fn fn-type="equal" id="FN1">
<label>*</label>
<p>M.G. and I.M.W. contributed equally to this work.</p>
</fn>
<fn fn-type="other" id="FN2">
<label></label>
<p>C.P. and M.S.C. are co–senior authors.</p>
</fn>
</author-notes>
<pub-date pub-type="ppub">
<day>10</day>
<month>1</month>
<year>2013</year>
</pub-date>
<pub-date pub-type="epreprint">
<day>13</day>
<month>11</month>
<year>2012</year>
</pub-date>
<pub-date pub-type="pmc-release">
<day>10</day>
<month>1</month>
<year>2014</year>
</pub-date>
<pmc-comment> PMC Release delay is 12 months and 0 days and was based on the . </pmc-comment>
<volume>121</volume>
<issue>2</issue>
<fpage>278</fpage>
<lpage>285</lpage>
<history>
<date date-type="received">
<day>30</day>
<month>4</month>
<year>2012</year>
</date>
<date date-type="accepted">
<day>11</day>
<month>10</month>
<year>2012</year>
</date>
</history>
<permissions>
<copyright-statement>© 2013 by The American Society of Hematology</copyright-statement>
<copyright-year>2013</copyright-year>
</permissions>
<self-uri xlink:title="pdf" xlink:type="simple" xlink:href="zh800213000278.pdf"></self-uri>
<abstract>
<p>Mediastinal large B-cell lymphoma (MLBL) represents 2% of mature B-cell non-Hodgkin lymphoma in patients ≤ 18 years of age. We analyzed data from childhood and adolescent patients with stage III MLBL (n = 42) and non-MLBL DLBCL (n = 69) treated with Group B therapy in the French-American-British/Lymphome Malins de Burkitt (FAB/LMB) 96 study. MLBL patients had a male/female 26/16; median age, 15.7 years (range, 12.5-19.7); and LDH < 2 versus ≥ 2 × the upper limit of normal, 23:19. Six MLBL patients (14%) had < a 20% response to initial COP (cyclophosphamide, vincristine, and prednisone) therapy. Central pathology revealed approximately 50% with classical features of primary MLBL. Five-year event-free survival for the stage III MLBL and non-MLBL DLBCL groups was 66% (95% confidence interval [CI], 49%-78%) and 85% (95% CI, 71%-92%), respectively (
<italic>P</italic>
< .001; 14%). The 5-year overall survival in the 42 MLBL patients was 73% (95% CI, 56%-84%). We conclude that MLBL in adolescent patients is associated with significantly inferior event-free survival compared with stage III non-MLBL DLBCL and can be of multiple histologies. Alternate treatment strategies should be investigated in the future taking into account both adult MLBL approaches and more recent biologic findings in adult MLBL.</p>
</abstract>
<abstract abstract-type="executive-summary">
<title>Key Points</title>
<p>
<list list-type="bullet">
<list-item>
<p>There are multiple histologic types of MLBCL in adolescents.</p>
</list-item>
<list-item>
<p>Event-free survival of MLBCL in adolescents after FAB/LMB 96 therapy is less compared to DLBCL.</p>
</list-item>
</list>
</p>
</abstract>
<funding-group>
<award-group>
<funding-source id="CS100">National Institutes of Health</funding-source>
</award-group>
</funding-group>
</article-meta>
</front>
</pmc>
</record>

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