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

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)...

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Main Authors: Ya K Mangasarova, A U Magomedova, S K Kravchenko, E E Zvonkov, A M Kremenetskaya, V I Vorobyev, D S Maryin, A V Gubkin, N I Skidan, I B Kaplanskaya, I A Vorobyev, R S Samoilova, A I Vorobyev
Format: Article
Language:Russian
Published: "Consilium Medicum" Publishing house 2010-07-01
Series:Терапевтический архив
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Online Access:https://ter-arkhiv.ru/0040-3660/article/view/30640
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author Ya K Mangasarova
A U Magomedova
S K Kravchenko
E E Zvonkov
A M Kremenetskaya
V I Vorobyev
D S Maryin
A V Gubkin
N I Skidan
I B Kaplanskaya
I A Vorobyev
R S Samoilova
A I Vorobyev
author_facet Ya K Mangasarova
A U Magomedova
S K Kravchenko
E E Zvonkov
A M Kremenetskaya
V I Vorobyev
D S Maryin
A V Gubkin
N I Skidan
I B Kaplanskaya
I A Vorobyev
R S Samoilova
A I Vorobyev
author_sort Ya K Mangasarova
collection DOAJ
description 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.
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spelling doaj.art-98b37fd343c64390b1ece2c909c551c52022-12-21T19:40:09Zrus"Consilium Medicum" Publishing houseТерапевтический архив0040-36602309-53422010-07-01827616527667Diffuse large B-cell lymphoma with primary involvement of mediastinal lymph nodes: diagnosis and treatmentYa K Mangasarova0A U Magomedova1S K Kravchenko2E E Zvonkov3A M Kremenetskaya4V I Vorobyev5D S Maryin6A V Gubkin7N I Skidan8I B Kaplanskaya9I A Vorobyev10R S Samoilova11A I Vorobyev12Hematology Research Center, Russian Academy of Medical SciencesHematology Research Center, Russian Academy of Medical SciencesHematology Research Center, Russian Academy of Medical SciencesHematology Research Center, Russian Academy of Medical SciencesHematology Research Center, Russian Academy of Medical SciencesHematology Research Center, Russian Academy of Medical SciencesHematology Research Center, Russian Academy of Medical SciencesHematology Research Center, Russian Academy of Medical SciencesHematology Research Center, Russian Academy of Medical SciencesHematology Research Center, Russian Academy of Medical SciencesHematology Research Center, Russian Academy of Medical SciencesHematology Research Center, Russian Academy of Medical SciencesHematology Research Center, Russian Academy of Medical SciencesAim. 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.https://ter-arkhiv.ru/0040-3660/article/view/30640mnhl-bfm-90diffuse large b-cell lymphosarcomaprimary mediastinal lymphosarcomamnhl-bfm-90radiotherapy
spellingShingle Ya K Mangasarova
A U Magomedova
S K Kravchenko
E E Zvonkov
A M Kremenetskaya
V I Vorobyev
D S Maryin
A V Gubkin
N I Skidan
I B Kaplanskaya
I A Vorobyev
R S Samoilova
A I Vorobyev
Diffuse large B-cell lymphoma with primary involvement of mediastinal lymph nodes: diagnosis and treatment
Терапевтический архив
mnhl-bfm-90
diffuse large b-cell lymphosarcoma
primary mediastinal lymphosarcoma
mnhl-bfm-90
radiotherapy
title Diffuse large B-cell lymphoma with primary involvement of mediastinal lymph nodes: diagnosis and treatment
title_full Diffuse large B-cell lymphoma with primary involvement of mediastinal lymph nodes: diagnosis and treatment
title_fullStr Diffuse large B-cell lymphoma with primary involvement of mediastinal lymph nodes: diagnosis and treatment
title_full_unstemmed Diffuse large B-cell lymphoma with primary involvement of mediastinal lymph nodes: diagnosis and treatment
title_short Diffuse large B-cell lymphoma with primary involvement of mediastinal lymph nodes: diagnosis and treatment
title_sort diffuse large b cell lymphoma with primary involvement of mediastinal lymph nodes diagnosis and treatment
topic mnhl-bfm-90
diffuse large b-cell lymphosarcoma
primary mediastinal lymphosarcoma
mnhl-bfm-90
radiotherapy
url https://ter-arkhiv.ru/0040-3660/article/view/30640
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