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  • 1 Országos Onkológiai Intézet Malignus Lymphoma Központ Budapest Ráth György u. 7–9. 1122
  • 2 Országos Onkológiai Intézet Sebészi és Molekuláris Patológiai Osztály Budapest
  • 3 Országos Onkológiai Intézet Radioterápiás Osztály Budapest
  • 4 Országos Onkológiai Intézet Radiodiagnosztikai Osztály Budapest
  • 5 Országos Onkológiai Intézet Epidemiológiai Osztály Budapest
  • 6 Semmelweis Egyetem, Általános Orvostudományi Kar I. Patológiai és Kísérletes Rákkutató Intézet Budapest
  • 7 Pozitron Diagnosztikai Kft. Budapest
Open access

A primer mediastinalis lymphoma (PMBCL) az agresszív diffúz nagy B-sejtes lymphomák (DLBCL) közé tartozó, ritka, jellegzetes klinikai képpel járó megbetegedés. A betegségre a mediastinalis lokalizáció, a gyors növekedési tendencia, a környező szövetekbe, szervekbe való infiltratív terjedés jellemző. A betegek kétharmada fiatal nő. Retrospektív vizsgálatok alapján az úgynevezett harmadik generációs kezelések (procarbazin-prednisolon-doxorubicin-cyclophosphamid-etoposid-cytosin-arabinosid-bleomycin-vincristin-methotrexat [ProMACE-CytaBOM], methotrexat-doxorubicin-cyclophosphamid-vincristin-prednisolon-bleomycin [MACOP-B], etoposid-doxorubicin-cyclophosphamid-vincristin-prednisolon-bleomycin [VACOP-B]) a korábbi standard cyclophosphamid-doxorubicin-vincristin-prednisolon (CHOP) kezelésnél hatékonyabbnak bizonyultak. Célok: A szerzők célja annak megállapítása, hogy a standard CHOP-kezeléshez adott anti-CD20 monoklonális antitest, a rituximab, javítja-e a kezelés hatékonyságát, a korábban általuk alkalmazott CHOP és harmadik generációs kezeléssel összevetve. Módszerek: 2002. október és 2004. december között 20 újonnan diagnosztizált, kezeletlen PMBCL-s beteg rituximab-CHOP (R-CHOP) kezelését kezdték. Az eredményeket a korábbi években CHOP (n = 9), illetve ProMACE-CytaBOM kombinációval (n = 15) kezelt 24 beteg eredményével hasonlították össze. Eredmények: Az átlag 64,6 hónapos követési idő során az ötéves teljes túlélés (OS) az R-CHOP-csoportban a CHOP-vel kezeltekéhez képest szignifikánsan kedvezőbbnek bizonyult (79,4% vs. 33,3%; p = 0,026). A kis esetszám miatt azonban az ötéves eseménymentes (EFS: 70,0% vs. 33,3%; p>0,05), betegségmentes (DFS: 70,0% vs. 33,3%; p>0,05) és relapsusmentes túlélésben (RFS: 93,0% vs. 100%; p>0,05) statisztikai különbség a nagy számszerű eltérés ellenére sem volt kimutatható. Az R-CHOP- és a ProMACE-CytaBOM kezelés adatait összevetve, hasonló eredményeket kaptak, statisztikai különbség közöttük nem volt kimutatható (OS: 79,4% vs. 80%; EFS: 70,0% vs. 60,0%; DFS: 70,0% vs. 60,0%; RFS: 93,0% vs. 82,0%; a p-érték minden esetben >0,05). Következtetések: A szerzők PMBCL-ben korábban nem kellő hatékonyságúnak tartott standard CHOP-kezelés rituximabbal történő kiegészítésével igen jó terápiás hatást értek el. A betegek az R-CHOP-kezeléseket jelentősebb mellékhatások és szövődmények nélkül, döntően „járó betegként” kapták. A DLBCL kezelésében standard R-CHOP-kezelés így a toxikusabb kezeléseket a PMBCL-ben is kiválthatja. Az eredmények a nemzetközi irodalmi adatokkal összevethetők. Orv. Hetil., 2011, 152, 735–742.

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  • Faris, J. E., LaCasce, A. S.: Primary mediastinal large B-cell lymphoma. Clin. Adv. Hematol. Oncol., 2009, 7, 125–133.

    LaCasce A. S. , 'Primary mediastinal large B-cell lymphoma ' (2009 ) 7 Clin. Adv. Hematol. Oncol. : 125 -133.

    • Search Google Scholar
  • Rodriquez, J., Gitiérrez, A., Piris, M.: Primary mediastinal B-cell lymphoma: treatment and therapeutic targets. Leuk. Lymphoma, 2008, 49, 1050–1061.

    Piris M. , 'Primary mediastinal B-cell lymphoma: treatment and therapeutic targets ' (2008 ) 49 Leuk. Lymphoma : 1050 -1061.

    • Search Google Scholar
  • Rohatiner, A. Z. S., Whelan, J. S., Ganjoo, R. K. és mtsai: Mediastinal large-cell lymphoma with sclerosis (MLCLS). Br. J. Cancer, 1994, 69, 601–604.

    Ganjoo R. K. , 'Mediastinal large-cell lymphoma with sclerosis (MLCLS) ' (1994 ) 69 Br. J. Cancer : 601 -604.

    • Search Google Scholar
  • Tzankov, A., Pehrs, A. C., Zimpfer, A. és mtsai: Prognostic significance of CD44 expression in diffuse large B cell lymphoma of activated and germinal centre B cell-like types: a tissue microarray analysis of 90 cases. J. Clin. Pathol., 2003, 56, 747–752.

    Zimpfer A. , 'Prognostic significance of CD44 expression in diffuse large B cell lymphoma of activated and germinal centre B cell-like types: a tissue microarray analysis of 90 cases ' (2003 ) 56 J. Clin. Pathol. : 747 -752.

    • Search Google Scholar
  • Von Basien, K., Kelta, M. és Bahaguna, P.: Primary mediastinal B-cell lymphoma: a reviev of pathology and management. J. Clin. Oncol., 2001, 19, 1855–1864.

    Bahaguna P. , 'Primary mediastinal B-cell lymphoma: a reviev of pathology and management ' (2001 ) 19 J. Clin. Oncol. : 1855 -1864.

    • Search Google Scholar
  • Swerdlow, S. H., Campo, E., Harris, N. L. és mtsai: WHO classification of tumours of haematopoietic and lymphoid tissues. 4th ed. IARC Press, Lion, France, 2008.

    Harris N. L. , '', in WHO classification of tumours of haematopoietic and lymphoid tissues , (2008 ) -.

    • Search Google Scholar
  • Savage, K. J., Monti, S., Kutok, J. L. és mtsai: The molecular signature of mediastinal B-cell lymphoma differs from that of other diffuse large B-cell lymphomas and shares features with classical Hodgkin lymphoma. Blood, 2003, 102, 3871–3879.

    Kutok J. L. , 'The molecular signature of mediastinal B-cell lymphoma differs from that of other diffuse large B-cell lymphomas and shares features with classical Hodgkin lymphoma ' (2003 ) 102 Blood : 3871 -3879.

    • Search Google Scholar
  • Rosenwald, A., Wright, G., Leroy, K. és mtsai: Molecular diagnosis of primary mediastinal B cell lymphoma identifies a clinically favorable subgroup of diffuse large B cell lymphoma related to Hodgkin lymphoma. J. Exp. Med., 2003, 198, 851–862.

    Leroy K. , 'Molecular diagnosis of primary mediastinal B cell lymphoma identifies a clinically favorable subgroup of diffuse large B cell lymphoma related to Hodgkin lymphoma ' (2003 ) 198 J. Exp. Med. : 851 -862.

    • Search Google Scholar
  • Shipp, M. A., Ross, K. N., Tamayo, P. és mtsai: Diffuse large B-cell lymphoma outcome prediction by gene-expression profiling and supervised machine learning. Nature Med., 2002, 8, 68–74.

    Tamayo P. , 'Diffuse large B-cell lymphoma outcome prediction by gene-expression profiling and supervised machine learning ' (2002 ) 8 Nature Med. : 68 -74.

    • Search Google Scholar
  • Staudt, L. M.: Molecular diagnosis of the hematologic cancers. N. Engl. J. Med., 2003, 348, 1777–1785.

    Staudt L. M. , 'Molecular diagnosis of the hematologic cancers ' (2003 ) 348 N. Engl. J. Med. : 1777 -1785.

    • Search Google Scholar
  • Wright, G., Tan, B., Rosenwald, A. és mtsai: A gen expression-based method to diagnose clinically distinct subgroups of diffuse large B cell lymphoma. Proc. Nat. Acad. Sci., 2003, 100, 9991–9996.

    Rosenwald A. , 'A gen expression-based method to diagnose clinically distinct subgroups of diffuse large B cell lymphoma ' (2003 ) 100 Proc. Nat. Acad. Sci. : 9991 -9996.

    • Search Google Scholar
  • Avilés, A., García, E. L., Fernández, R. és mtsai: Combined therapy in the treatment of primary mediastinal B-cell lymphoma: conventional versus escalated chemotherapy. Ann. Hematol., 2002, 81, 368–373.

    Fernández R. , 'Combined therapy in the treatment of primary mediastinal B-cell lymphoma: conventional versus escalated chemotherapy ' (2002 ) 81 Ann. Hematol. : 368 -373.

    • Search Google Scholar
  • Cazals-Hatem, D., Lepage, E., Brice, P. és mtsai: Primary mediastinal large B-cell lymphoma. Clinicopathologic study of 141 cases compared with 916 nonmediastinal large B-cell lymphomas, a GELA (“Groupe d’Etude des Lymphomes de l’Adulte) study. Am. J. Surg. Pathol., 1996, 20, 877–888.

    Brice P. , 'Primary mediastinal large B-cell lymphoma. Clinicopathologic study of 141 cases compared with 916 nonmediastinal large B-cell lymphomas, a GELA (“Groupe d’Etude des Lymphomes de l’Adulte) study ' (1996 ) 20 Am. J. Surg. Pathol. : 877 -888.

    • Search Google Scholar
  • Lazzaino, M., Orlandi, E., Pauli, M. és mtsai: Treatment outcome and prognostic factors for primary mediastinal (thymic) B-cell lymphoma: a multicenter study of 106 patients. J. Clin. Oncol., 1997, 15, 1646–1653.

    Pauli M. , 'Treatment outcome and prognostic factors for primary mediastinal (thymic) B-cell lymphoma: a multicenter study of 106 patients ' (1997 ) 15 J. Clin. Oncol. : 1646 -1653.

    • Search Google Scholar
  • Nguyen, L. N., Ha, C. S., Hess, M. és mtsai: The outcome of combined-modality treatments for stage I and II primary large B-cell lymphoma of the mediastinum. Int. J. Rad. Oncol. Biol. Phys., 2000, 15, 1281–1285.

    Hess M. , 'The outcome of combined-modality treatments for stage I and II primary large B-cell lymphoma of the mediastinum ' (2000 ) 15 Int. J. Rad. Oncol. Biol. Phys. : 1281 -1285.

    • Search Google Scholar
  • Popat, U., Przepiork, D., Chanplin, R. és mtsai: High-dose chemotherapy for relapsed and refractory diffuse large B-cell lymphoma: mediastinal localisation predict for a favorable outcome. J. Clin. Oncol., 1998, 16, 63–69.

    Chanplin R. , 'High-dose chemotherapy for relapsed and refractory diffuse large B-cell lymphoma: mediastinal localisation predict for a favorable outcome ' (1998 ) 16 J. Clin. Oncol. : 63 -69.

    • Search Google Scholar
  • Sehn, L. H., Antin, J. H., Shulman, L. N. és mtsai: Primary diffuse large B-cell lymphoma of the mediastinum: outcome following high-dose chemotherapy and autologous hematopoietic cell transplantation. Blood, 1998, 91, 717–723.

    Shulman L. N. , 'Primary diffuse large B-cell lymphoma of the mediastinum: outcome following high-dose chemotherapy and autologous hematopoietic cell transplantation ' (1998 ) 91 Blood : 717 -723.

    • Search Google Scholar
  • Shipp, M. A., Abeloff, K. H., Caroll, G. és mtsai: International consensus conferenceon high-dose therapy with haematopoietic stem cell transplantation in aggressive non-Hodgkin’s lymphomas: Report of the jury. J. Clin. Oncol., 1999, 17, 423–429.

    Caroll G. , 'International consensus conferenceon high-dose therapy with haematopoietic stem cell transplantation in aggressive non-Hodgkin’s lymphomas: Report of the jury ' (1999 ) 17 J. Clin. Oncol. : 423 -429.

    • Search Google Scholar
  • Massoud, M., Koscielny, S., Lapusan, S. és mtsai: Primary mediastinal large B-cell lymphomas treated with dose-intensified CHOP alone or CHOP combined with radiotherapy. Leuk. Lymphoma, 2008, 49, 1510–1515.

    Lapusan S. , 'Primary mediastinal large B-cell lymphomas treated with dose-intensified CHOP alone or CHOP combined with radiotherapy ' (2008 ) 49 Leuk. Lymphoma : 1510 -1515.

    • Search Google Scholar
  • Mazzarotto, R., Boso, K., Vianello, F. és mtsai: Primary mediastinal large B-cell lymphoma: results of intenzive chemotherapy regiments (MACOP-B/VACOP-B) plus involved field radiotherapy on 53 patients. A single institution experience. Int. J. Rad. Oncol. Biol. Phys., 2007, 68, 823–829.

    Vianello F. , 'Primary mediastinal large B-cell lymphoma: results of intenzive chemotherapy regiments (MACOP-B/VACOP-B) plus involved field radiotherapy on 53 patients. A single institution experience ' (2007 ) 68 Int. J. Rad. Oncol. Biol. Phys. : 823 -829.

    • Search Google Scholar
  • Savage, K. J., Al-Rajhi, N., Voss, N. és mtsai: Favorable outcome of primary mediastinal large B-cell lymphoma in a single institution: the British Columbia experience. Ann. Oncol., 2006, 17, 123–130.

    Voss N. , 'Favorable outcome of primary mediastinal large B-cell lymphoma in a single institution: the British Columbia experience ' (2006 ) 17 Ann. Oncol. : 123 -130.

    • Search Google Scholar
  • Zinzani, P. L., Martelli, M., de Renzo, A. és mtsai: Primary mediastinal large B-cell lymphoma with sclerosis: a clinical study of 89 patients treated with MACOP-B chemotherapy and radiation therapy. Haematologica, 2001, 86, 187–191.

    Renzo A. , 'Primary mediastinal large B-cell lymphoma with sclerosis: a clinical study of 89 patients treated with MACOP-B chemotherapy and radiation therapy ' (2001 ) 86 Haematologica : 187 -191.

    • Search Google Scholar
  • Coiffier, B., Lepage, E., Briere, J. és mtsai: CHOP chemotherapy plus rituximab compared with CHOP alone in elderly patients with diffuse large-B-cell lymphoma. N. Engl. J. Med., 2002, 346, 235–242.

    Briere J. , 'CHOP chemotherapy plus rituximab compared with CHOP alone in elderly patients with diffuse large-B-cell lymphoma ' (2002 ) 346 N. Engl. J. Med. : 235 -242.

    • Search Google Scholar
  • Pfreundschuh, M., Trümper, L., Österborg, A. és mtsai: CHOP-like chemotherapy plus rituximab versus CHOP-like chemotherapy alone in young patients with good-prognosis diffuse large-B-cell lymphoma: a randomised controlled trial by MabThera International Trial (MInT) group. Lancet Oncol., 2006, 7, 379–391.

    Österborg A. , 'CHOP-like chemotherapy plus rituximab versus CHOP-like chemotherapy alone in young patients with good-prognosis diffuse large-B-cell lymphoma: a randomised controlled trial by MabThera International Trial (MInT) group ' (2006 ) 7 Lancet Oncol. : 379 -391.

    • Search Google Scholar
  • Shen, L. H., Donaldson, J., Chhanabhai, M. és mtsai: Introduction of combined CHOP plus rituximab therapy dramatically improved outcome of diffuse large B-cell lymphoma in British Columbia. J. Clin. Oncol., 2005, 23, 5027–5033.

    Chhanabhai M. , 'Introduction of combined CHOP plus rituximab therapy dramatically improved outcome of diffuse large B-cell lymphoma in British Columbia ' (2005 ) 23 J. Clin. Oncol. : 5027 -5033.

    • Search Google Scholar
  • Zinzani, P. L., Stefoni, V., Finolezzi, E. és mtsai: Rituximab combined with MACOP-B or VACOP-B and radiation therapy in primary mediastinal large B-cell lymphoma: a retrospective study. Clin. Lymphoma Myeloma, 2009, 9, 381–385.

    Finolezzi E. , 'Rituximab combined with MACOP-B or VACOP-B and radiation therapy in primary mediastinal large B-cell lymphoma: a retrospective study ' (2009 ) 9 Clin. Lymphoma Myeloma : 381 -385.

    • Search Google Scholar
  • Dunleavy, K., Pittaluga, S., Janik, J. és mtsai: Primary mediastinal large B-cell lymphoma (PMBL) outcome is significantly improved by the addition of rituximab to dose adjusted (DA)-EPOCH and overcomes the need for radiation. Blood, 2005, 106, Abstract, 929.

    Janik J. , 'Primary mediastinal large B-cell lymphoma (PMBL) outcome is significantly improved by the addition of rituximab to dose adjusted (DA)-EPOCH and overcomes the need for radiation ' (2005 ) 106 Blood : 929 -.

    • Search Google Scholar
  • Schneider, T., Molnár, Zs., Tóth, E. és mtsai: Excellent results in the treatment of primary mediastinal lymphomas. Blood, 2010, 116, Abstract, 4915.

    Tóth E. , 'Excellent results in the treatment of primary mediastinal lymphomas ' (2010 ) 116 Blood : 4915 -.

    • Search Google Scholar
  • Schneider, T., Orosz, Zs., Ésik, O. és mtsai: Primary mediastinal non-Hodgkin’s lymphoma. Hematol. J., 2000, 1 (Suppl. 1), Abstract, 695.

    Ésik O. , 'Primary mediastinal non-Hodgkin’s lymphoma ' (2000 ) 1 Hematol. J. : 695 -.

  • Schneider T., Tóth E., Molnár Zs. és mtsai: Primer mediastinalis nagy B-sejtes lymphomák kezelése. Orv. Hetil., 2004, 145, 2531–2537.

    Molnár Zs. , 'Primer mediastinalis nagy B-sejtes lymphomák kezelése ' (2004 ) 145 Orv. Hetil. : 2531 -2537.

    • Search Google Scholar
  • Cheson, B. D., Horning, S. J., Coiffier, B. és mtsai: Report of an International Workshop to standardized response criteria for non-Hodgkin´s lymphomas. J. Clin. Oncol., 1999, 17, 1244–1253.

    Coiffier B. , 'Report of an International Workshop to standardized response criteria for non-Hodgkin´s lymphomas ' (1999 ) 17 J. Clin. Oncol. : 1244 -1253.

    • Search Google Scholar
  • Kaplan, E. L., Meier, P.: Nonparametric estimation from incomplete observations. J. Am. Stat. Assoc., 1958, 58, 457–481.

    Meier P. , 'Nonparametric estimation from incomplete observations ' (1958 ) 58 J. Am. Stat. Assoc. : 457 -481.

    • Search Google Scholar
  • Eichelmann, A., Koretz, K., Mechterscheimer, G. és mtsa: Adhesion receptor profile of thymic B-cell lymphoma. Am. J. Pathol., 1992, 141, 729–741.

    Mechterscheimer G. , 'Adhesion receptor profile of thymic B-cell lymphoma ' (1992 ) 141 Am. J. Pathol. : 729 -741.

    • Search Google Scholar
  • Joos, S., Otano-Joos, M. I., Ziegler, S. és mtsai: Primary mediastinal (thymic) B-cell lymphoma is characterized by gains of chromosomal material including 9p and amplication of the REL gene. Blood, 1996, 87, 1571–1578.

    Ziegler S. , 'Primary mediastinal (thymic) B-cell lymphoma is characterized by gains of chromosomal material including 9p and amplication of the REL gene ' (1996 ) 87 Blood : 1571 -1578.

    • Search Google Scholar
  • Fischer, R. I., Gaynor, E. R., Dahlberg, S. és mtsai: Comparison of a standard regimen (CHOP) with three intensive chemotherapy regiments for advanced non-Hodgkin’s lymphoma. N. Engl. J. Med., 1993, 328, 1002–1006.

    Dahlberg S. , 'Comparison of a standard regimen (CHOP) with three intensive chemotherapy regiments for advanced non-Hodgkin’s lymphoma ' (1993 ) 328 N. Engl. J. Med. : 1002 -1006.

    • Search Google Scholar

 

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