IKBFU's Vestnik

2019 Issue № 1

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Case study: treatment of a patient with Т- cell lymphoma (delta-gamma-variant)



In this article, we present a clinical case of a patient with T-cell lympho­ma (delta-gamma variant) complicated by neuroleukemia. We performed a dif­ferential diagnosis from other lymphoproliferative diseases. Our diagnosis was based on trepanobiopsy and immunophenotyping data: delta-gamma-T/NK-cell lymphoma, hepatolienal form. The first NHL-BFM 90 protocol block was obtained. We performed a whole body PET/CT scan and a myelogram to con­firm the diagnosis. Since therapy was ineffective, we conducted two courses of second-line therapy (R-ICE) and received a partial response. Further, alloge­neic hematopoietic stem cell transplantation was recommended. After two R-ICE courses, the patient received supportive polychemotherapy (Rituximab + + Gem­zar), without progression. Six months later, the patient's condition wors­ened. We performed spinal puncture and diagnosed neuroleukemia. It was recom­mended that the patient received a specific course of polychemother­apy. There­fore, patients diagnosed with hepatolienal T-cell lymphoma, in view of the highly aggressive disease course, are recommended to undergo allogene­ic trans­plantation of hematopoietic stem cells as early as possible.


1. Крячок И. А., Филоненко Е. С. Особенности Т-клеточных лимфом // Вест­ник гематологии. 2014. Т. 10, № 1. С. 48—55.

2. Swerdlow S. H., Campo E., Harris N. L. et al. WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues. Lyon, 2008.

3. Виноградова Ю. Е., Зингерман Б. В. Нозологические формы и выживаемость пациентов с Т- и НК-клеточными лимфатическими опухолями, наблюдавших­ся в ГНЦ в течение 10 лет // Клиническая онкогематология. 2011. № 4 (3). С. 201—212.

4. Chiang A. K., Srivastava G., Lau P. W., Ho F. C. Differences in T-cell receptor gene rearrangement and transcription in nasal lymphomas of natural killer and T-cell types: implications on cellular origin // Hum Pathol. 1996. № 27. P. 701—707.

5. Reinherz E. L., Kung P. C., Goldstein G. et al. Discrete stages of human in­trathymic differentiation: analysis of normal thymocytes and leukemic lymphoblasts of T-cell lineage // Proc Nat Acad Sci. 1980. Vol. 77 (3). P. 1588—1592.

6. Джалилов А. Ф. Неходжкинские лимфомы: основы классификации и им­муноцитохимической диагностики // Онкология. 2013. № 15 (4). C. 264—272.

7. Доронин В. А. Т-клеточные лимфомы: возможности терапии при ограни­ченном выборе // Клиническая онкогематология. 2013. № 6 (2). C. 127—138.

8. Schmitz N., Trumper L., Ziepert M. et al. Treatment and prognosis of mature T-cell and NK-cell lymphoma: an analysis of patients with T-cell lymphoma treated in studies of the German High-Grade Non-Hodgkin Lymphoma Study Group // Blood. 2010. Vol. 116. C. 3418—3425.

9. Gallamini A., Zaja F., Patti C. et al. Alemtuzumab (Campath-1H) and CHOP сhemotherapy as first-line treatment of peripheral T-cell lymphoma: results of a GITIL (Gruppo Italiano Terapie Innovative nei Linfomi) prospective multicenter trial // Blood. 2007. Vol. 110. P. 2316—2323.

10. Mahadevan D., Unger J. M., Persky D. O. et al. Phase II trial of cisplatin plus etoposide plus gemcitagine plus solumedrol (PEGS) in peripheral T-cell non-Hodg­kin lymphoma (SWOG S0350) // Ann Oncol. 2011. Vol. 22 (4). P. 119—120.

11. Rodriguez J., Conde E., Gutierrez A. et al. Frontline autologous stem cell trans­plantation in high-risk peripheral T-cell lymphoma: a prospective study from The Gel-Tamo Study Group // Eur J Haematol. 2007. Vol. 79 (1). P. 32—38.

12. Mercadal S. Intensive chemotherapy (high-dose CHOP/ESHAP regimen) fol­lowed by autologous stem-cell transplantation in previously untreated patients with peripheral T-cell lymphoma // Ann Oncol. 2008. Vol. 19 (5). P. 958—963.

13. Reimer P., Rudiger T., Geissinger E. et al. Autologous stem-cell transplantation as first-line therapy in peripheral T-cell lymphomas: results of a prospective multi­center study // J Clin Oncol. 2009. Vol. 27 (1). P. 106—113.

14. d’Amore F., Relander T., Lauritzsen G. F. et al. High-dose chemotherapy and au­tologuos stem cell transplantation in previously untreated peripheral T-cell lym­phoma — final analysis of a large prospective multicenter study (NLG-T-01) // J Clin Oncol. 2012. Vol. 30 (25). P. 3093—3099.

15. Ghazal M. A. Hepatosplenic T-cell lymphoma: case report & literature review // Hematology and Transfusion International Journal. 2017. Vol. 5 (1). P. 191‒193.

16. Чернова Н. Г., Джулакян У. Л., Виноградова Ю. Е. и др. Гепатолиенальная 
Т-клеточная лимфома: проблемы диагностики и лечения // Терапевтический архив. 2016.  № 88 (7). C. 4—14.

17. Yabe M., Medeiros L. J., Tang G. et al. Prognostic factors of hepatosplenic T-cell lymphoma clinicopathologic study of 28 cases // American Journal of Surgical Pa­thology 2016. Vol. 40 (5). P. 676—688.

18. Falchook G. S., Vega F., Dang N. H. et al. Hepatosplenic gamma-delta T-cell lym­phoma: clinicopathological features and treatment // Ann Oncol. 2009. Vol. 20 (6). P. 1080—1085.

19. Weidmann E. Hepatosplenic T cell lymphoma. A review on 45 cases since the first report describing the disease as a distinct lymphoma entity in 1990 // Leuke­mia. 2000. Vol. 14 (6). P. 991—997.