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


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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.

About the authors

Ya K Mangasarova

Hematology Research Center, Russian Academy of Medical Sciences

Email: v.k.jana@mail.ru
Hematology Research Center, Russian Academy of Medical Sciences

A U Magomedova

Hematology Research Center, Russian Academy of Medical Sciences

Email: maminat@mail.ru
Hematology Research Center, Russian Academy of Medical Sciences

S K Kravchenko

Hematology Research Center, Russian Academy of Medical Sciences

Email: krav@blood.ru
Hematology Research Center, Russian Academy of Medical Sciences

E E Zvonkov

Hematology Research Center, Russian Academy of Medical Sciences

Email: zvonkov@blood.ru
Hematology Research Center, Russian Academy of Medical Sciences

A M Kremenetskaya

Hematology Research Center, Russian Academy of Medical Sciences

Email: mtkalexandra@gmail.com
Hematology Research Center, Russian Academy of Medical Sciences

V I Vorobyev

Hematology Research Center, Russian Academy of Medical Sciences

Email: morela@mail.ru
Hematology Research Center, Russian Academy of Medical Sciences

D S Maryin

Hematology Research Center, Russian Academy of Medical Sciences

Email: dmdoc@mail.ru
Hematology Research Center, Russian Academy of Medical Sciences

A V Gubkin

Hematology Research Center, Russian Academy of Medical Sciences

Email: gubkin@blood.ru
Hematology Research Center, Russian Academy of Medical Sciences

N I Skidan

Hematology Research Center, Russian Academy of Medical Sciences

Email: nskidan@rabmler.ru
Hematology Research Center, Russian Academy of Medical Sciences

I B Kaplanskaya

Hematology Research Center, Russian Academy of Medical Sciences

Email: irinak@blood.ru
Hematology Research Center, Russian Academy of Medical Sciences

I A Vorobyev

Hematology Research Center, Russian Academy of Medical Sciences

Email: ivorobjev@mail.ru
Hematology Research Center, Russian Academy of Medical Sciences

R S Samoilova

Hematology Research Center, Russian Academy of Medical Sciences

Email: tb@blood.ru
Hematology Research Center, Russian Academy of Medical Sciences

A I Vorobyev

Hematology Research Center, Russian Academy of Medical Sciences

Email: director@blood.ru
Hematology Research Center, Russian Academy of Medical Sciences

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Copyright (c) 2010 Mangasarova Y.K., Magomedova A.U., Kravchenko S.K., Zvonkov E.E., Kremenetskaya A.M., Vorobyev V.I., Maryin D.S., Gubkin A.V., Skidan N.I., Kaplanskaya I.B., Vorobyev I.A., Samoilova R.S., Vorobyev A.I.

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