[Diagnosis and treatment in patients with B-cell lymphoma unclassified that is intermediate between diffuse large B-cell lymphoma and Burkitt's lymphoma].

Abstract:

AIM:To characterize a group of patients with B-cell lymphoma (BCLU) unclassified that is intermediate between diffuse large B-cell lymphoma and Burkitt's lymphoma, to identify poor prognostic factors, and to evaluate therapeutic efficiency in patients with BCLU. SUBJECTS AND METHODS:Twenty-five patients with BCLU were examined. Double-hit lymphoma (DHL) was diagnosed in 8 (32%) patients. According to the Ann-Arbor classification of lymphoma, its stages II, III, and IV were diagnosed in 3 (12%), 2 (8%), and 20 (80%) patients, respectively. MYC rearrangement was observed in 11 (48%) out of 23 patients: single-hit lymphoma in 3 patients and DHL in 8 (BCL2+/MYC+ in 6 cases and BCL6+/MYC+ in 2). The expression of с-MYC (cut off ≥40%) was revealed in 17 (74%) out of 23 patients; that of BCL2 (cut off ≥50%) was detected in 14 (58%) out of 24 patients; coexpression of both proteins was seen in 12 (52%) out of 23 patients. The DHL group showed a correlation between the rearrangement of the BCL2+/MYC+ genes and the expression of MYC and BCL2 proteins in 5 out of 6 patients. Taking into account the heterogeneity of the entire patient group, DHL and non-DHL subgroups were considered separately. Both subgroups were comparable by clinical characteristics. BCLU patients younger than 60 years of age received treatment according to the LB-M-04 ± rituximab; those aged 60 or older had CHOP-like regimens ± rituximab. Autologous stem cell transplantation (auto-SCT) was performed in 5 patients belonging to a high-risk group. RESULTS:The 3-year overall survival (OS) was 62% and the 3-year event-free survival (EFS) was 51%. The 3-year OS was lower for the DHL group than that for the non-DHL group (43 and 75%, respectively). CONCLUSION:In the DHL group, both OS and EFS are significantly lower (the risk of poor outcome, including death, is higher) than those in the non-DHL group. It is conceivable that intensified chemotherapy with auto-SCT increases treatment results in patients with BCLU; however, a larger number of observations are needed to obtain valid data. :Цель исследования. Охарактеризовать группу больных неклассифицируемой В-клеточной лимфомой (ВНЛ), занимающей промежуточное положение между диффузной В-крупноклеточной лимфомой (ДВККЛ) и лимфомой Беркитта (ЛБ), выделить факторы неблагоприятного прогноза и оценить эффективность терапии больных ВНЛ. Материалы и методы. Обследовали 25 больных ВНЛ. 'Double-hit' лимфома (DHL) диагностирована у 8 (32%) больных. Согласно классификации Ann-Arbor II стадия заболевания диагностирована у 3 (12%) больных, III - у 2 (8%), IV - у 20 (80%). Перестройка локуса гена MYC наблюдалась у 11 (48%) из 23 пациентов: 'single-hit' - у 3, DHL - у 8 (у 6 BCL2+/MYC+, у 2 BCL6+/MYC+). Экспрессия с-MYC (пороговый уровень ≥40%) выявлялась у 17 (74%) из 23 больных, экспрессия BCL2 (пороговый уровень ≥50%) - у 14 (58%) из 24, коэкспрессия обоих белков - у 12 (52%) из 23. В группе DHL корреляция перестройки генов BCL2+/MYC+ и экспрессии белков BCL2+/MYC+ наблюдалась у 5 из 6 пациентов. Учитывая гетерогенность общей группы пациентов, подгруппы больных DHL и non-DHL рассматривали отдельно. Обе группы были сопоставимы по клиническим характеристикам. Больным ВНЛ моложе 60 лет проводили терапию согласно протоколу ЛБ-М-04 ± ритуксимаб, пациентам старше 60 лет - CHOP-подобными курсами ± ритуксимаб. Трансплантацию аутологичных стволовых клеток крови (ауто-ТСКК) выполняли 5 больным, относящихся к группе высокого риска. Результаты. 3-летняя общая выживаемость (ОВ) составила 62%, 3-летняя бессобытийная выживаемость (БСВ) - 51%. 3-летняя ОВ для группы больных c DHL оказалась ниже, чем у больных non-DHL, - 43 и 75% соответственно. Заключение. Как ОВ, так и БСВ у пациентов c DHL существенно ниже (а риск неблагоприятного исхода, включая смерть, выше), чем у пациентов группы non-DHL. Возможно, интенсификация терапии c ауто-ТСКК повышает эффективность лечения пациентов с ВНЛ, однако для получения достоверных данных необходимо большее количество наблюдений.

journal_name

Ter Arkh

journal_title

Terapevticheskii arkhiv

authors

Baryakh EA,Misyurina AE,Kovrigina AM,Obukhova TN,Gemdzhyan EG,Vorobyev VI,Mangasarova YK,Polyakov YY,Magomedova AU,Klyasova GA,Misyurin VA,Yatsyk GA,Shevelev AA,Kostina IE,Vorobyev AI,Kravchenko SK

doi

10.17116/terarkh201587877-85

subject

Has Abstract

pub_date

2015-01-01 00:00:00

pages

77-85

issue

8

eissn

0040-3660

issn

2309-5342

journal_volume

87

pub_type

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