Diffuse large B-cell lymphoma with primary involvement of mediastinal lymph nodes: diagnosis and treatment

Ya K Mangasarova; Мангасарова Яна Константиновна; A U Magomedova; Магомедова Аминат Умарасхабовна; S K Kravchenko; Кравченко Сергей Кириллович; E E Zvonkov; Звонков Евгений Евгеньевич; A M Kremenetskaya; Кременецкая Александра Михайловна; V I Vorobyev; Воробьев Владимир Иванович; D S Maryin; Марьин Дмитрий Сергеевич; A V Gubkin; Губкин Андрей Владимирович; N I Skidan; Скидан Наталья Ивановна; I B Kaplanskaya; Капланская Ирина Борисовна; I A Vorobyev; Воробьев Иван Андреевич; R S Samoilova; Самойлова Римма Семеновна; A I Vorobyev; Воробьев Андрей Иванович

Diffuse large B-cell lymphoma with primary involvement of mediastinal lymph nodes: diagnosis and treatment

Authors: Mangasarova Y.K¹, Magomedova AU¹, Kravchenko SK¹, Zvonkov EE¹, Kremenetskaya AM¹, Vorobyev VI¹, Maryin DS¹, Gubkin AV¹, Skidan NI¹, Kaplanskaya IB¹, Vorobyev IA¹, Samoilova RS¹, Vorobyev AI¹
Affiliations:
1. Hematology Research Center, Russian Academy of Medical Sciences
Issue: Vol 82, No 7 (2010)
Pages: 61-65
Section: Editorial
URL: https://ter-arkhiv.ru/0040-3660/article/view/30640
ID: 30640

Cite item

Abstract

Aim. To diagnose diffuse large B-cell lymphosarcoma (DLBCLS) with primary involvement of the mediastinal lymph nodes (LN) and to evaluate the efficiency of aggressive polychemotherapy (PCT).
Subjects and methods. The study included 15 patients (6 men and 9 women aged 18 to 70 years; median 38 years) followed up at the Hematology Research Center, Russian Academy of Medical Sciences, in 2004 to 2009. Three and 12 patients had Stages II and IIE DLBCLS, respectively. B symptoms were found in 14 (93.4%) patients. Increased lactate dehydrogenase (LDH) concentrations were detectable in 14 (93.4%) patients; tumors of 10 cm or more (bulky disease) were seen in 11 (73.3%). Enlarged cervical, supraclavicular, and axillary lymph nodes were found in 9 (60%) patients; lung involvement via extension in 9 (60%), and invasion into the pericardium in 5 (33.3%) and soft tissues of the anterior thoracic wall in (13.3%). There were no signs of involvement of extranodal organs at the moment of diagnosis. All the 15 patients received PCT according to the modified NHL-BFM-90 program: 4 to 6 courses depending on the response to the therapy; 10 (66.6%) and 5 (33.3%) patients had 4 and 6 courses, respectively; for consolidating purpose, 11 (78.5%) patients were prescribed radiotherapy applied to the mediastinum in a cumulative dose of 36 Gy due to the fact that they had a residual mass.
Results. Thirteen (86.6%) patients achieved a complete remission (CR). Primary PCT resistance was confirmed in one case. Another patient was stated to have near-complete remission. No recurrences were notified during the follow-up. The mean CR duration was 24.5 (range 2-49) months.
Conclusion. DLBCLS with primary LN involvement is an individual nosological entity to be differentiated from primary mediastinal large B-cell lymphosarcoma. In most cases, DLBCLS shows signs of a poor prognosis, which makes it necessary to perform aggressive PCT.

Keywords

mNHL-BFM-90, diffuse large B-cell lymphosarcoma, primary mediastinal lymphosarcoma, mNHL-BFM-90, radiotherapy

References

Swerdlow S. H., Campo E. et al. Diffuse large B-cell lymphoma, not otherwise specified. In: WHO classification of hematopoetic and lymphoid tissues. Lyon; 2008. 233-237.
Isaacson P. G., Norton A., Addis B. The human thymus contains a novel population of B-lymphocytes. Lancet 1987; 2: 1488-1491.
Bertran H. C., Check I. J., Milano M. A. Immunophenotyping large B-cell lymphomas flow cytometric pitfalls and pathologic correlation. Am. J. Clin. Pathol. 2001; 116: 191-203.
Loddeenkemper C., Anagnostopoulos I., Hummel M. et al. Differential Emu enhancer activity expression of BOB.1/OBF.1, Oct2, PU.1, and immunoglobulin in reactive B-cell populations, B-cell non-Hodgkin lymphomas, and Hodgkin lymphomas. J. Pathol. 2004: 202; 60-69.
Winter J. N., Weller E. A., Horning S. J. et al. Prognostic significance of bcl-6 protein expression in DLBCL treated with CHOP or R-CHOP: prospective correlative study. Blood. 2006; 107 (11): 4207-4213.
Barrans S., Evans P., Connor S. et al. The t(14;18) is associated with germinal center-derived diffuse B-cell lymphoma and is a strong predictor. Clin. Cancer Res. 2003; 9: 2133-2139.
Ковригина А. М., Пробатова Н. А. Лимфома Ходжкина и крупноклеточные лимфомы. М.: МИА; 2007. 108-124.
Leithauser F., Bauerle M., Huynh M. Q. et al. Isotype-switched immunoglobulin genes with a high load of somatic hypermutation and lasc of ongoing mutation activity are prelevant in mediastinal B-cell lymphoma. Blood 2001; 98: 2762-2770.
Copie- Bergman C., Gaulard P., Maouche-Chrétien L. et al. The MAL gene is expressed in primary mediastinal large B-cell lymphoma. Blood 1999; 94: 3567-3575.
Barans S., Connor S., Evans P. et al. Rearrangement of the BCL-6 locus at 3q27 is an independent poor prognostic factor in nodal diffuse large B-cell lymphoma. Br. J. Haematol. 2002; 117: 322-332.
Магомедова А. У. Диффузная В-крупноклеточная лимфосаркома лимфоидных органов: клинические формы, лечение. Автореф. дис. ... д-ра мед. наук. М.; 2008.
Bentz M., Barth T., Bruderlein S. et al. Gain of chromosome arm 9p is characteristic of primary mediastinal B-cell lymphoma (MBL): Comprehensive molecular cytogenetic analysis and presentation of a novel MBL cell line. Genes, Chromos. Cancer 2001; 30: 393-401.
Feugier P., Van Hoof A., Sebban S. et al. Long-term results of the R-CHOP study in the treatment of elderly patients with diffuse Large B-cell lymphoma: A Study by the Groupe d'Etude des Lymphomes de l'Adulte. J. Clin. Oncol. 2005; 23: 4117-4126.
Поддубная В. И., Османов Д. Ш., Бабичева Л. Г. и др. Сравнительная характеристика эффективности схем СНОР и Мабтера + СНОР (R-CHOP) в лечении больных диффузной крупноклеточной В-клеточной неходжкинской лимфомой. Соврем. онкол. 2004; 6: 39-41.
Звонков Е. Е., Кременецкая А. М., Кравченко С. К. и др. Эффективность консервативной терапии лимфосаркомы желудка. Тер. арх. 2008; 7: 18-26.
Магомедова А. У., Кравченко С. К., Кременецкая А. М. и др. Модифицированная программа NHL-BFM-90 в лечении больных диффузной В-крупноклеточной лимфосаркомой. Тер. арх. 2006; 10: 44-47.
Барях Е. А., Звонков Е. Е., Кременецкая А. М. и др. Лечение беркиттоподобной лимфомы взрослых. Тер. арх. 2005; 8: 53-57.
Магомедова А. У., Кравченко С. К., Кременецкая А. М. Диффузная В-крупноклеточная лимфосаркома. Диагностические критерии, алгоритм обследования и основные программы лечения. Пробл. гематол. 2004; 2: 26-28.
Zinzani P. L., Martelli M., Bertini M. et al. 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- 1264.
Cheson B. D., Pfistner B., Juweid M. E. et al. Revised response criteria for malignant lymphoma. J. Clin. Oncol. 2007; 25 (5): 579-586.

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Diffuse large B-cell lymphoma with primary involvement of mediastinal lymph nodes: diagnosis and treatment

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