Own experience of primary mediastinal B-large cell lymphoma treatment

Due to the relative low incidence of primary mediastinal B-large cell lymphoma (PM BLCL) optimal approaches to its treatment is still not well developed. Possibility to improve PM BLCL clinical outcomes by intensifying induction chemotherapy (CT) and the use of rituximab, the usefulness of high-CT with autologous stem cells transplantation (autologous HSCT) and radiotherapy (RT) is currently being studied.
The purpose of this study was to evaluate the efficacy and tolerability of induction therapy MACOP-B with or without rituximab in patients with PM BLCL. 34 patients with PM BLCL, received MACOP-B (n = 10) or R-MACOP-B (n = 24) in P.A. Herzen Moscow Research Institute of Oncology during January 2006 and August 2013, were included in the study. 28 patients (82.4 %) achieved partial/complete remission after MACOP-B ± rituximab completing. In case of insufficient response (large residual tumor) or primary resistance patients received the second line chemotherapy and/or autologous HSCT. 25 patients received radiotherapy for residual tumor mass after CT completion. After completion of full treatment program remission was achieved in total of 32 patients (94.1 %): complete remission in 27 (79.4 %) and partial remission in 5 (14.7 %). Relapse occurred in 3 patients (8.8 %). With a median follow-up of 36.5 months, 3-year disease-free survival was 93 %, eventfree survival – 75 % and overall survival – 90 %. Thus, the “MACOP-B ± rituximab” program was highly effective and acceptable tolerated in PM BLCL patients. The necessity of auto-HSCT and radiotherapy remains debatable.

1. Lichtenstein A. K., Levine A., Taylor C. R. et al. Primary mediastinal lymphoma in adults. Am J Med 1980;68:509–14.

2. Gaulard P., Harris N. L., Pileri S. A. et al. Primary mediastinal (thymic) large B-cell lymphoma. In: World Health Organization Classification of Tumours: Pathology and Genetics of Tumours of Haematopoietic and Lymphoid Tissues (Jaffe E. S., Harris N. L., Stein H., Vardiman J. W., eds.). IARC Press Lyon, France, 2008. Pp. 250–251.

3. Lazzarino M., Orlandi E., Paulli M. et al. Treatment outcome and prognostic factors for primary mediastinal (thymic) B-cell lymphoma: a multicenter study of 106 patients. J Clin Oncol 1997;15:1646–53.

4. Aisenberg A. C. Primary large-cell lymphoma of the mediastinum. J Clin Oncol 1993;11:2291–4.

5. Zinzani P. L., Martelli M., Bertini M. et al. International Extranodal Lymphoma Study Group (IELSG). Induction chemotherapy strategies for primary mediastinal large B-cell lymphoma with sclerosis: a retrospective multinational study on 426 previously untreated patients. Haematologica 2002;87:1258–64.

6. Todeschini G., Secchi S., Morra E. et al. Primary mediastinal large B-cell lymphoma (PMLBCL): longterm results from a retrospective multicentre Italian experience in 138 patients treated with CHOP or MACOP-B/VACOP-B. Br J Cancer 2004;90:372–6.

7. Savage K. J., Al-Rajhi N., Voss N. et al. Favorable outcome of primary mediastinal large B-cell lymphoma in a single institution: the British Columbia experience. Ann Oncol 2006;17:123–30.

8. Cartron G., Watier H., Golay J. and Solal- Celigny P. From the bench to the bedside: ways to improve rituximab efficacy. Blood 2004;104:2635–42.

9. Zinzani P. L., Stefoni V., Finolezzi E. et al. 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;9:381–5.

10. Dunleavy K., Pittaluga S., Janik J. et al. 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;106:929.

11. Dunleavy K., Pittaluga S., Janik J. et al. Primary mediastinal large B-cell lymphoma (PMBL) outcome may be significantly improved by the addition of rituximab to dose-adjusted (DA) – EPOCH and оbviates the need for radiation: results from a prospective study of 44 patients. Blood 2006;108:209.

12. Xu L. M., Fang H., Wang W. H. et al. Prognostic significance of rituximab and radiotherapy for patients with primary mediastinal large B-cell lymphoma receiving doxorubicin-containing chemotherapy. Leuk Lymphoma 2013;54 (8):1684–90.

13. Avigdor А., Sirotkin T., Shemtov N. et al. Combination of rituximab with initial chemotherapy improves the outcome of patients with primary mediastinal В-cell lymphoma: a retrospective analysis of a single institution cohort. Haematologica 2008;93:307.

14. Rodríguez J., Conde E., Gutiérrez A. et al. Primary mediastinal large cell lymphoma (PMBL): frontline treatment with autologous stem cell transplantation (ASCT). The GEL-TAMO experience. Hematol Oncol 2008;26:171–8.