Primary CNS Posttransplant Lymphoproliferative Disease (PTLD): An International Report of 84 Cases in the Modern Era
Identifieur interne : 000704 ( PascalFrancis/Checkpoint ); précédent : 000703; suivant : 000705Primary CNS Posttransplant Lymphoproliferative Disease (PTLD): An International Report of 84 Cases in the Modern Era
Auteurs : A. M. Evens [États-Unis] ; S. Choquet [France] ; A. R. Kroll-Desrosiers [États-Unis] ; D. Jagadeesh [États-Unis] ; S. M. Smith [États-Unis] ; F. Morschhauser [France] ; V. Leblond [France] ; R. Roy [États-Unis] ; B. Barton [États-Unis] ; L. I. Gordon [États-Unis] ; M. K. Gandhi [Australie] ; D. Dierickx [Belgique] ; D. Schiff [États-Unis] ; T. M. Habermann [États-Unis] ; R. Trappe [Allemagne]Source :
- American journal of transplantation : (Print) [ 1600-6135 ] ; 2013.
Descripteurs français
- Pascal (Inist)
- Syndrome lymphoprolifératif posttransplantation, Primaire, Système nerveux central, Syndrome lymphoprolifératif, International, Monde, Lymphome, Compte rendu, Etude cas, Rituximab, Pronostic, Immunodépresseur, Immunothérapie, Anticorps monoclonal, Anticancéreux, Immunomodulateur, Traitement, Antigène CD20, Hémopathie maligne lymphoïde.
English descriptors
- KwdEn :
- Antineoplastic agent, Case study, Central nervous system, Immunomodulator, Immunosuppressive agent, Immunotherapy, International, Lymphoid neoplasm, Lymphoma, Lymphoproliferative syndrome, Monoclonal antibody, Posttransplant lymphoproliferative disorder, Primary, Prognosis, Report, Rituximab, Treatment, World.
Abstract
We performed a multicenter, International analysis of solid organ transplant (SOT)-related primary central nervous system (PCNS) posttransplant lymphoproliferative disease (PTLD). Among 84 PCNS PTLD patients, median time of SOT-to-PTLD was 54 months, 79% had kidney SOT, histology was monomorphic in 83% and tumor was EBV+ in 94%. Further, 33% had deep brain involvement, 10% had CSF involvement, while none had ocular disease. Immunosuppression was reduced in 93%; additional first-line therapy included high-dose methotrexate (48%), high-dose cytarabine (33%), brain radiation (24%) and/or rituximab (44%). The overall response rate was 60%, while treatment-related mortality was 13%. With 42-month median follow-up, three-year progression-free survival (PFS) and overall survival (OS) were 32% and 43%, respectively. There was a trend on univariable analysis for improved PFS for patients who received rituximab and/or high-dose cytarabine. On multivariable Cox regression, poor performance status predicted inferior PFS (HR 2.61, 95% CI 1.32-5.17, p = 0.006), while increased LDH portended inferior OS (HR 4.16, 95% CI 1.29-13.46, p = 0.02). Moreover, lack of response to first-line therapy was the most dominant prognostic factor on multivariable analysis (HR 8.70, 95% CI 2.56- 29.57, p = 0.0005). Altogether, PCNS PTLD appears to represent a distinct clinicopathologic entity within the PTLD spectrum that is associated with renal SOT, occurs late, is monomorphic and retains EBV positivity.
Affiliations:
- Allemagne, Australie, Belgique, France, États-Unis
- Massachusetts, Schleswig-Holstein, Île-de-France
- Amherst (Massachusetts), Kiel, Paris
- Université du Massachusetts
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M. Smith</name><affiliation wicri:level="1"><inist:fA14 i1="04"><s1>Departament of Medicine, Section of Hematology/Oncology, University of Chicago Hospitals</s1><s2>Chicago, IL</s2><s3>USA</s3><sZ>5 aut.</sZ></inist:fA14><country>États-Unis</country><wicri:noRegion>Chicago, IL</wicri:noRegion></affiliation></author><author><name sortKey="Morschhauser, F" sort="Morschhauser, F" uniqKey="Morschhauser F" first="F." last="Morschhauser">F. Morschhauser</name><affiliation wicri:level="3"><inist:fA14 i1="05"><s1>Hematology, Lille University Hospital</s1><s2>Paris</s2><s3>FRA</s3><sZ>6 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="Leblond, V" sort="Leblond, V" uniqKey="Leblond V" first="V." last="Leblond">V. Leblond</name><affiliation wicri:level="3"><inist:fA14 i1="02"><s1>Hematology, Hospital Pitie-Salpetriere</s1><s2>Paris</s2><s3>FRA</s3><sZ>2 aut.</sZ><sZ>7 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="Roy, R" sort="Roy, R" uniqKey="Roy R" first="R." last="Roy">R. Roy</name><affiliation wicri:level="1"><inist:fA14 i1="06"><s1>Division of Hematology/Oncology and Robert H. Lurie Comprehensive Cancer Center, Feinberg School of Medicine, Northwestern University</s1><s2>Chicago, IL</s2><s3>USA</s3><sZ>8 aut.</sZ><sZ>10 aut.</sZ></inist:fA14><country>États-Unis</country><wicri:noRegion>Chicago, IL</wicri:noRegion></affiliation></author><author><name sortKey="Barton, B" sort="Barton, B" uniqKey="Barton B" first="B." last="Barton">B. Barton</name><affiliation wicri:level="4"><inist:fA14 i1="03"><s1>Ouantitative Health Sciences, The University of Massachusetts</s1><s2>Worcester, MA</s2><s3>USA</s3><sZ>3 aut.</sZ><sZ>9 aut.</sZ></inist:fA14><country>États-Unis</country><placeName><settlement type="city">Amherst (Massachusetts)</settlement><region type="state">Massachusetts</region></placeName><orgName type="university">Université du Massachusetts</orgName></affiliation></author><author><name sortKey="Gordon, L I" sort="Gordon, L I" uniqKey="Gordon L" first="L. I." last="Gordon">L. I. Gordon</name><affiliation wicri:level="1"><inist:fA14 i1="06"><s1>Division of Hematology/Oncology and Robert H. Lurie Comprehensive Cancer Center, Feinberg School of Medicine, Northwestern University</s1><s2>Chicago, IL</s2><s3>USA</s3><sZ>8 aut.</sZ><sZ>10 aut.</sZ></inist:fA14><country>États-Unis</country><wicri:noRegion>Chicago, IL</wicri:noRegion></affiliation></author><author><name sortKey="Gandhi, M K" sort="Gandhi, M K" uniqKey="Gandhi M" first="M. K." last="Gandhi">M. K. Gandhi</name><affiliation wicri:level="1"><inist:fA14 i1="07"><s1>Princess Alexandra Hospital</s1><s2>Brisbane, Old</s2><s3>AUS</s3><sZ>11 aut.</sZ></inist:fA14><country>Australie</country><wicri:noRegion>Princess Alexandra Hospital</wicri:noRegion></affiliation></author><author><name sortKey="Dierickx, D" sort="Dierickx, D" uniqKey="Dierickx D" first="D." last="Dierickx">D. Dierickx</name><affiliation wicri:level="1"><inist:fA14 i1="08"><s1>Department of Hematology, University Hospitals Leuven</s1><s2>Leuven</s2><s3>BEL</s3><sZ>12 aut.</sZ></inist:fA14><country>Belgique</country><wicri:noRegion>Leuven</wicri:noRegion></affiliation></author><author><name sortKey="Schiff, D" sort="Schiff, D" uniqKey="Schiff D" first="D." last="Schiff">D. Schiff</name><affiliation wicri:level="1"><inist:fA14 i1="09"><s1>Division of Neuro-Oncology, University of Virginia</s1><s2>Charlottesville, VA</s2><s3>USA</s3><sZ>13 aut.</sZ></inist:fA14><country>États-Unis</country><wicri:noRegion>Charlottesville, VA</wicri:noRegion></affiliation></author><author><name sortKey="Habermann, T M" sort="Habermann, T M" uniqKey="Habermann T" first="T. M." last="Habermann">T. M. Habermann</name><affiliation wicri:level="1"><inist:fA14 i1="10"><s1>Division of Hematology, Mayo Clinic</s1><s2>Rochester, MN</s2><s3>USA</s3><sZ>14 aut.</sZ></inist:fA14><country>États-Unis</country><wicri:noRegion>Rochester, MN</wicri:noRegion></affiliation></author><author><name sortKey="Trappe, R" sort="Trappe, R" uniqKey="Trappe R" first="R." last="Trappe">R. Trappe</name><affiliation wicri:level="3"><inist:fA14 i1="11"><s1>Department of Hematology and Oncology, University Medical Center Schleswig-Holstein, Campus Kiel</s1><s2>Kiel</s2><s3>DEU</s3><sZ>15 aut.</sZ></inist:fA14><country>Allemagne</country><placeName><region type="land" nuts="2">Schleswig-Holstein</region><settlement type="city">Kiel</settlement></placeName></affiliation></author></titleStmt><publicationStmt><idno type="wicri:source">INIST</idno><idno type="inist">13-0235169</idno><date when="2013">2013</date><idno type="stanalyst">PASCAL 13-0235169 INIST</idno><idno type="RBID">Pascal:13-0235169</idno><idno type="wicri:Area/PascalFrancis/Corpus">000953</idno><idno type="wicri:Area/PascalFrancis/Curation">005523</idno><idno type="wicri:Area/PascalFrancis/Checkpoint">000704</idno><idno type="wicri:explorRef" wicri:stream="PascalFrancis" wicri:step="Checkpoint">000704</idno></publicationStmt><sourceDesc><biblStruct><analytic><title xml:lang="en" level="a">Primary CNS Posttransplant Lymphoproliferative Disease (PTLD): An International Report of 84 Cases in the Modern Era</title><author><name sortKey="Evens, A M" sort="Evens, A M" uniqKey="Evens A" first="A. M." last="Evens">A. M. Evens</name><affiliation wicri:level="1"><inist:fA14 i1="01"><s1>Division of Hematology/Oncology, The University of Massachusetts Medical School</s1><s2>Worcester, MA</s2><s3>USA</s3><sZ>1 aut.</sZ><sZ>4 aut.</sZ></inist:fA14><country>États-Unis</country><wicri:noRegion>Worcester, MA</wicri:noRegion></affiliation></author><author><name sortKey="Choquet, S" sort="Choquet, S" uniqKey="Choquet S" first="S." last="Choquet">S. Choquet</name><affiliation wicri:level="3"><inist:fA14 i1="02"><s1>Hematology, Hospital Pitie-Salpetriere</s1><s2>Paris</s2><s3>FRA</s3><sZ>2 aut.</sZ><sZ>7 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="Kroll Desrosiers, A R" sort="Kroll Desrosiers, A R" uniqKey="Kroll Desrosiers A" first="A. R." last="Kroll-Desrosiers">A. R. Kroll-Desrosiers</name><affiliation wicri:level="4"><inist:fA14 i1="03"><s1>Ouantitative Health Sciences, The University of Massachusetts</s1><s2>Worcester, MA</s2><s3>USA</s3><sZ>3 aut.</sZ><sZ>9 aut.</sZ></inist:fA14><country>États-Unis</country><placeName><settlement type="city">Amherst (Massachusetts)</settlement><region type="state">Massachusetts</region></placeName><orgName type="university">Université du Massachusetts</orgName></affiliation></author><author><name sortKey="Jagadeesh, D" sort="Jagadeesh, D" uniqKey="Jagadeesh D" first="D." last="Jagadeesh">D. Jagadeesh</name><affiliation wicri:level="1"><inist:fA14 i1="01"><s1>Division of Hematology/Oncology, The University of Massachusetts Medical School</s1><s2>Worcester, MA</s2><s3>USA</s3><sZ>1 aut.</sZ><sZ>4 aut.</sZ></inist:fA14><country>États-Unis</country><wicri:noRegion>Worcester, MA</wicri:noRegion></affiliation></author><author><name sortKey="Smith, S M" sort="Smith, S M" uniqKey="Smith S" first="S. M." last="Smith">S. M. Smith</name><affiliation wicri:level="1"><inist:fA14 i1="04"><s1>Departament of Medicine, Section of Hematology/Oncology, University of Chicago Hospitals</s1><s2>Chicago, IL</s2><s3>USA</s3><sZ>5 aut.</sZ></inist:fA14><country>États-Unis</country><wicri:noRegion>Chicago, IL</wicri:noRegion></affiliation></author><author><name sortKey="Morschhauser, F" sort="Morschhauser, F" uniqKey="Morschhauser F" first="F." last="Morschhauser">F. Morschhauser</name><affiliation wicri:level="3"><inist:fA14 i1="05"><s1>Hematology, Lille University Hospital</s1><s2>Paris</s2><s3>FRA</s3><sZ>6 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="Leblond, V" sort="Leblond, V" uniqKey="Leblond V" first="V." last="Leblond">V. Leblond</name><affiliation wicri:level="3"><inist:fA14 i1="02"><s1>Hematology, Hospital Pitie-Salpetriere</s1><s2>Paris</s2><s3>FRA</s3><sZ>2 aut.</sZ><sZ>7 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="Roy, R" sort="Roy, R" uniqKey="Roy R" first="R." last="Roy">R. Roy</name><affiliation wicri:level="1"><inist:fA14 i1="06"><s1>Division of Hematology/Oncology and Robert H. Lurie Comprehensive Cancer Center, Feinberg School of Medicine, Northwestern University</s1><s2>Chicago, IL</s2><s3>USA</s3><sZ>8 aut.</sZ><sZ>10 aut.</sZ></inist:fA14><country>États-Unis</country><wicri:noRegion>Chicago, IL</wicri:noRegion></affiliation></author><author><name sortKey="Barton, B" sort="Barton, B" uniqKey="Barton B" first="B." last="Barton">B. Barton</name><affiliation wicri:level="4"><inist:fA14 i1="03"><s1>Ouantitative Health Sciences, The University of Massachusetts</s1><s2>Worcester, MA</s2><s3>USA</s3><sZ>3 aut.</sZ><sZ>9 aut.</sZ></inist:fA14><country>États-Unis</country><placeName><settlement type="city">Amherst (Massachusetts)</settlement><region type="state">Massachusetts</region></placeName><orgName type="university">Université du Massachusetts</orgName></affiliation></author><author><name sortKey="Gordon, L I" sort="Gordon, L I" uniqKey="Gordon L" first="L. I." last="Gordon">L. I. Gordon</name><affiliation wicri:level="1"><inist:fA14 i1="06"><s1>Division of Hematology/Oncology and Robert H. Lurie Comprehensive Cancer Center, Feinberg School of Medicine, Northwestern University</s1><s2>Chicago, IL</s2><s3>USA</s3><sZ>8 aut.</sZ><sZ>10 aut.</sZ></inist:fA14><country>États-Unis</country><wicri:noRegion>Chicago, IL</wicri:noRegion></affiliation></author><author><name sortKey="Gandhi, M K" sort="Gandhi, M K" uniqKey="Gandhi M" first="M. K." last="Gandhi">M. K. Gandhi</name><affiliation wicri:level="1"><inist:fA14 i1="07"><s1>Princess Alexandra Hospital</s1><s2>Brisbane, Old</s2><s3>AUS</s3><sZ>11 aut.</sZ></inist:fA14><country>Australie</country><wicri:noRegion>Princess Alexandra Hospital</wicri:noRegion></affiliation></author><author><name sortKey="Dierickx, D" sort="Dierickx, D" uniqKey="Dierickx D" first="D." last="Dierickx">D. Dierickx</name><affiliation wicri:level="1"><inist:fA14 i1="08"><s1>Department of Hematology, University Hospitals Leuven</s1><s2>Leuven</s2><s3>BEL</s3><sZ>12 aut.</sZ></inist:fA14><country>Belgique</country><wicri:noRegion>Leuven</wicri:noRegion></affiliation></author><author><name sortKey="Schiff, D" sort="Schiff, D" uniqKey="Schiff D" first="D." last="Schiff">D. Schiff</name><affiliation wicri:level="1"><inist:fA14 i1="09"><s1>Division of Neuro-Oncology, University of Virginia</s1><s2>Charlottesville, VA</s2><s3>USA</s3><sZ>13 aut.</sZ></inist:fA14><country>États-Unis</country><wicri:noRegion>Charlottesville, VA</wicri:noRegion></affiliation></author><author><name sortKey="Habermann, T M" sort="Habermann, T M" uniqKey="Habermann T" first="T. M." last="Habermann">T. M. Habermann</name><affiliation wicri:level="1"><inist:fA14 i1="10"><s1>Division of Hematology, Mayo Clinic</s1><s2>Rochester, MN</s2><s3>USA</s3><sZ>14 aut.</sZ></inist:fA14><country>États-Unis</country><wicri:noRegion>Rochester, MN</wicri:noRegion></affiliation></author><author><name sortKey="Trappe, R" sort="Trappe, R" uniqKey="Trappe R" first="R." last="Trappe">R. Trappe</name><affiliation wicri:level="3"><inist:fA14 i1="11"><s1>Department of Hematology and Oncology, University Medical Center Schleswig-Holstein, Campus Kiel</s1><s2>Kiel</s2><s3>DEU</s3><sZ>15 aut.</sZ></inist:fA14><country>Allemagne</country><placeName><region type="land" nuts="2">Schleswig-Holstein</region><settlement type="city">Kiel</settlement></placeName></affiliation></author></analytic><series><title level="j" type="main">American journal of transplantation : (Print)</title><title level="j" type="abbreviated">Am. j. transplant. : (Print)</title><idno type="ISSN">1600-6135</idno><imprint><date when="2013">2013</date></imprint></series></biblStruct></sourceDesc><seriesStmt><title level="j" type="main">American journal of transplantation : (Print)</title><title level="j" type="abbreviated">Am. j. transplant. : (Print)</title><idno type="ISSN">1600-6135</idno></seriesStmt></fileDesc><profileDesc><textClass><keywords scheme="KwdEn" xml:lang="en"><term>Antineoplastic agent</term><term>Case study</term><term>Central nervous system</term><term>Immunomodulator</term><term>Immunosuppressive agent</term><term>Immunotherapy</term><term>International</term><term>Lymphoid neoplasm</term><term>Lymphoma</term><term>Lymphoproliferative syndrome</term><term>Monoclonal antibody</term><term>Posttransplant lymphoproliferative disorder</term><term>Primary</term><term>Prognosis</term><term>Report</term><term>Rituximab</term><term>Treatment</term><term>World</term></keywords><keywords scheme="Pascal" xml:lang="fr"><term>Syndrome lymphoprolifératif posttransplantation</term><term>Primaire</term><term>Système nerveux central</term><term>Syndrome lymphoprolifératif</term><term>International</term><term>Monde</term><term>Lymphome</term><term>Compte rendu</term><term>Etude cas</term><term>Rituximab</term><term>Pronostic</term><term>Immunodépresseur</term><term>Immunothérapie</term><term>Anticorps monoclonal</term><term>Anticancéreux</term><term>Immunomodulateur</term><term>Traitement</term><term>Antigène CD20</term><term>Hémopathie maligne lymphoïde</term></keywords></textClass></profileDesc></teiHeader><front><div type="abstract" xml:lang="en">We performed a multicenter, International analysis of solid organ transplant (SOT)-related primary central nervous system (PCNS) posttransplant lymphoproliferative disease (PTLD). Among 84 PCNS PTLD patients, median time of SOT-to-PTLD was 54 months, 79% had kidney SOT, histology was monomorphic in 83% and tumor was EBV+ in 94%. Further, 33% had deep brain involvement, 10% had CSF involvement, while none had ocular disease. Immunosuppression was reduced in 93%; additional first-line therapy included high-dose methotrexate (48%), high-dose cytarabine (33%), brain radiation (24%) and/or rituximab (44%). The overall response rate was 60%, while treatment-related mortality was 13%. With 42-month median follow-up, three-year progression-free survival (PFS) and overall survival (OS) were 32% and 43%, respectively. There was a trend on univariable analysis for improved PFS for patients who received rituximab and/or high-dose cytarabine. On multivariable Cox regression, poor performance status predicted inferior PFS (HR 2.61, 95% CI 1.32-5.17, p = 0.006), while increased LDH portended inferior OS (HR 4.16, 95% CI 1.29-13.46, p = 0.02). Moreover, lack of response to first-line therapy was the most dominant prognostic factor on multivariable analysis (HR 8.70, 95% CI 2.56- 29.57, p = 0.0005). Altogether, PCNS PTLD appears to represent a distinct clinicopathologic entity within the PTLD spectrum that is associated with renal SOT, occurs late, is monomorphic and retains EBV positivity.</div></front></TEI><inist><standard h6="B"><pA><fA01 i1="01" i2="1"><s0>1600-6135</s0></fA01><fA03 i2="1"><s0>Am. j. transplant. : (Print)</s0></fA03><fA05><s2>13</s2></fA05><fA06><s2>6</s2></fA06><fA08 i1="01" i2="1" l="ENG"><s1>Primary CNS Posttransplant Lymphoproliferative Disease (PTLD): An International Report of 84 Cases in the Modern Era</s1></fA08><fA11 i1="01" i2="1"><s1>EVENS (A. M.)</s1></fA11><fA11 i1="02" i2="1"><s1>CHOQUET (S.)</s1></fA11><fA11 i1="03" i2="1"><s1>KROLL-DESROSIERS (A. R.)</s1></fA11><fA11 i1="04" i2="1"><s1>JAGADEESH (D.)</s1></fA11><fA11 i1="05" i2="1"><s1>SMITH (S. M.)</s1></fA11><fA11 i1="06" i2="1"><s1>MORSCHHAUSER (F.)</s1></fA11><fA11 i1="07" i2="1"><s1>LEBLOND (V.)</s1></fA11><fA11 i1="08" i2="1"><s1>ROY (R.)</s1></fA11><fA11 i1="09" i2="1"><s1>BARTON (B.)</s1></fA11><fA11 i1="10" i2="1"><s1>GORDON (L. I.)</s1></fA11><fA11 i1="11" i2="1"><s1>GANDHI (M. K.)</s1></fA11><fA11 i1="12" i2="1"><s1>DIERICKX (D.)</s1></fA11><fA11 i1="13" i2="1"><s1>SCHIFF (D.)</s1></fA11><fA11 i1="14" i2="1"><s1>HABERMANN (T. M.)</s1></fA11><fA11 i1="15" i2="1"><s1>TRAPPE (R.)</s1></fA11><fA14 i1="01"><s1>Division of Hematology/Oncology, The University of Massachusetts Medical School</s1><s2>Worcester, MA</s2><s3>USA</s3><sZ>1 aut.</sZ><sZ>4 aut.</sZ></fA14><fA14 i1="02"><s1>Hematology, Hospital Pitie-Salpetriere</s1><s2>Paris</s2><s3>FRA</s3><sZ>2 aut.</sZ><sZ>7 aut.</sZ></fA14><fA14 i1="03"><s1>Ouantitative Health Sciences, The University of Massachusetts</s1><s2>Worcester, MA</s2><s3>USA</s3><sZ>3 aut.</sZ><sZ>9 aut.</sZ></fA14><fA14 i1="04"><s1>Departament of Medicine, Section of Hematology/Oncology, University of Chicago Hospitals</s1><s2>Chicago, IL</s2><s3>USA</s3><sZ>5 aut.</sZ></fA14><fA14 i1="05"><s1>Hematology, Lille University Hospital</s1><s2>Paris</s2><s3>FRA</s3><sZ>6 aut.</sZ></fA14><fA14 i1="06"><s1>Division of Hematology/Oncology and Robert H. Lurie Comprehensive Cancer Center, Feinberg School of Medicine, Northwestern University</s1><s2>Chicago, IL</s2><s3>USA</s3><sZ>8 aut.</sZ><sZ>10 aut.</sZ></fA14><fA14 i1="07"><s1>Princess Alexandra Hospital</s1><s2>Brisbane, Old</s2><s3>AUS</s3><sZ>11 aut.</sZ></fA14><fA14 i1="08"><s1>Department of Hematology, University Hospitals Leuven</s1><s2>Leuven</s2><s3>BEL</s3><sZ>12 aut.</sZ></fA14><fA14 i1="09"><s1>Division of Neuro-Oncology, University of Virginia</s1><s2>Charlottesville, VA</s2><s3>USA</s3><sZ>13 aut.</sZ></fA14><fA14 i1="10"><s1>Division of Hematology, Mayo Clinic</s1><s2>Rochester, MN</s2><s3>USA</s3><sZ>14 aut.</sZ></fA14><fA14 i1="11"><s1>Department of Hematology and Oncology, University Medical Center Schleswig-Holstein, Campus Kiel</s1><s2>Kiel</s2><s3>DEU</s3><sZ>15 aut.</sZ></fA14><fA20><s1>1512-1522</s1></fA20><fA21><s1>2013</s1></fA21><fA23 i1="01"><s0>ENG</s0></fA23><fA43 i1="01"><s1>INIST</s1><s2>27587</s2><s5>354000503626080160</s5></fA43><fA44><s0>0000</s0><s1>© 2013 INIST-CNRS. All rights reserved.</s1></fA44><fA45><s0>38 ref.</s0></fA45><fA47 i1="01" i2="1"><s0>13-0235169</s0></fA47><fA60><s1>P</s1></fA60><fA61><s0>A</s0></fA61><fA64 i1="01" i2="1"><s0>American journal of transplantation : (Print)</s0></fA64><fA66 i1="01"><s0>USA</s0></fA66><fC01 i1="01" l="ENG"><s0>We performed a multicenter, International analysis of solid organ transplant (SOT)-related primary central nervous system (PCNS) posttransplant lymphoproliferative disease (PTLD). Among 84 PCNS PTLD patients, median time of SOT-to-PTLD was 54 months, 79% had kidney SOT, histology was monomorphic in 83% and tumor was EBV+ in 94%. Further, 33% had deep brain involvement, 10% had CSF involvement, while none had ocular disease. Immunosuppression was reduced in 93%; additional first-line therapy included high-dose methotrexate (48%), high-dose cytarabine (33%), brain radiation (24%) and/or rituximab (44%). The overall response rate was 60%, while treatment-related mortality was 13%. With 42-month median follow-up, three-year progression-free survival (PFS) and overall survival (OS) were 32% and 43%, respectively. There was a trend on univariable analysis for improved PFS for patients who received rituximab and/or high-dose cytarabine. On multivariable Cox regression, poor performance status predicted inferior PFS (HR 2.61, 95% CI 1.32-5.17, p = 0.006), while increased LDH portended inferior OS (HR 4.16, 95% CI 1.29-13.46, p = 0.02). Moreover, lack of response to first-line therapy was the most dominant prognostic factor on multivariable analysis (HR 8.70, 95% CI 2.56- 29.57, p = 0.0005). Altogether, PCNS PTLD appears to represent a distinct clinicopathologic entity within the PTLD spectrum that is associated with renal SOT, occurs late, is monomorphic and retains EBV positivity.</s0></fC01><fC02 i1="01" i2="X"><s0>002B25</s0></fC02><fC02 i1="02" i2="X"><s0>002B19B</s0></fC02><fC03 i1="01" i2="X" l="FRE"><s0>Syndrome lymphoprolifératif posttransplantation</s0><s2>NM</s2><s5>01</s5></fC03><fC03 i1="01" i2="X" l="ENG"><s0>Posttransplant lymphoproliferative disorder</s0><s2>NM</s2><s5>01</s5></fC03><fC03 i1="01" i2="X" l="SPA"><s0>Síndrome linfoproliferativo postrasplante</s0><s2>NM</s2><s5>01</s5></fC03><fC03 i1="02" i2="X" l="FRE"><s0>Primaire</s0><s5>02</s5></fC03><fC03 i1="02" i2="X" l="ENG"><s0>Primary</s0><s5>02</s5></fC03><fC03 i1="02" i2="X" l="SPA"><s0>Primario</s0><s5>02</s5></fC03><fC03 i1="03" i2="X" l="FRE"><s0>Système nerveux central</s0><s5>03</s5></fC03><fC03 i1="03" i2="X" l="ENG"><s0>Central nervous system</s0><s5>03</s5></fC03><fC03 i1="03" i2="X" 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l="ENG"><s0>Treatment</s0><s5>27</s5></fC03><fC03 i1="17" i2="X" l="SPA"><s0>Tratamiento</s0><s5>27</s5></fC03><fC03 i1="18" i2="X" l="FRE"><s0>Antigène CD20</s0><s4>INC</s4><s5>86</s5></fC03><fC03 i1="19" i2="X" l="FRE"><s0>Hémopathie maligne lymphoïde</s0><s4>CD</s4><s5>96</s5></fC03><fC03 i1="19" i2="X" l="ENG"><s0>Lymphoid neoplasm</s0><s4>CD</s4><s5>96</s5></fC03><fC07 i1="01" i2="X" l="FRE"><s0>Hémopathie maligne</s0><s2>NM</s2><s5>37</s5></fC07><fC07 i1="01" i2="X" l="ENG"><s0>Malignant hemopathy</s0><s2>NM</s2><s5>37</s5></fC07><fC07 i1="01" i2="X" l="SPA"><s0>Hemopatía maligna</s0><s2>NM</s2><s5>37</s5></fC07><fC07 i1="02" i2="X" l="FRE"><s0>Cancer</s0><s2>NM</s2></fC07><fC07 i1="02" i2="X" l="ENG"><s0>Cancer</s0><s2>NM</s2></fC07><fC07 i1="02" i2="X" l="SPA"><s0>Cáncer</s0><s2>NM</s2></fC07><fC07 i1="03" i2="X" l="FRE"><s0>Anti-CD20</s0><s4>INC</s4><s5>87</s5></fC07><fN21><s1>217</s1></fN21><fN44 i1="01"><s1>OTO</s1></fN44><fN82><s1>OTO</s1></fN82></pA></standard></inist><affiliations><list><country><li>Allemagne</li><li>Australie</li><li>Belgique</li><li>France</li><li>États-Unis</li></country><region><li>Massachusetts</li><li>Schleswig-Holstein</li><li>Île-de-France</li></region><settlement><li>Amherst (Massachusetts)</li><li>Kiel</li><li>Paris</li></settlement><orgName><li>Université du Massachusetts</li></orgName></list><tree><country name="États-Unis"><noRegion><name sortKey="Evens, A M" sort="Evens, A M" uniqKey="Evens A" first="A. M." last="Evens">A. M. Evens</name></noRegion><name sortKey="Barton, B" sort="Barton, B" uniqKey="Barton B" first="B." last="Barton">B. Barton</name><name sortKey="Gordon, L I" sort="Gordon, L I" uniqKey="Gordon L" first="L. I." last="Gordon">L. I. Gordon</name><name sortKey="Habermann, T M" sort="Habermann, T M" uniqKey="Habermann T" first="T. M." last="Habermann">T. M. Habermann</name><name sortKey="Jagadeesh, D" sort="Jagadeesh, D" uniqKey="Jagadeesh D" first="D." last="Jagadeesh">D. Jagadeesh</name><name sortKey="Kroll Desrosiers, A R" sort="Kroll Desrosiers, A R" uniqKey="Kroll Desrosiers A" first="A. R." last="Kroll-Desrosiers">A. R. Kroll-Desrosiers</name><name sortKey="Roy, R" sort="Roy, R" uniqKey="Roy R" first="R." last="Roy">R. Roy</name><name sortKey="Schiff, D" sort="Schiff, D" uniqKey="Schiff D" first="D." last="Schiff">D. Schiff</name><name sortKey="Smith, S M" sort="Smith, S M" uniqKey="Smith S" first="S. M." last="Smith">S. M. Smith</name></country><country name="France"><region name="Île-de-France"><name sortKey="Choquet, S" sort="Choquet, S" uniqKey="Choquet S" first="S." last="Choquet">S. Choquet</name></region><name sortKey="Leblond, V" sort="Leblond, V" uniqKey="Leblond V" first="V." last="Leblond">V. Leblond</name><name sortKey="Morschhauser, F" sort="Morschhauser, F" uniqKey="Morschhauser F" first="F." last="Morschhauser">F. Morschhauser</name></country><country name="Australie"><noRegion><name sortKey="Gandhi, M K" sort="Gandhi, M K" uniqKey="Gandhi M" first="M. K." last="Gandhi">M. K. Gandhi</name></noRegion></country><country name="Belgique"><noRegion><name sortKey="Dierickx, D" sort="Dierickx, D" uniqKey="Dierickx D" first="D." last="Dierickx">D. Dierickx</name></noRegion></country><country name="Allemagne"><region name="Schleswig-Holstein"><name sortKey="Trappe, R" sort="Trappe, R" uniqKey="Trappe R" first="R." last="Trappe">R. 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