Hepatosplenic T-cell lymphoma: The problems of diagnosis and treatment

Chernova N.G.; Dzhulakian U.L.; Vinogradova Yu.E.; Sidorova Yu.V.; Ryzhikova N.V.; Korzhova S.M.; Sinitsyna M.N.; Tikhomirov D.S.; Naumova E.V.; Obukhova T.N.; Dvirnyk V.N.; Sudarikov A.B.; Kovrigina A.M.; Kravchenko S.K.; Melikjan A.L.; Kuz'mina L.A.; Gal'tseva I.V.; Smirnova S.Yu.; Gemdzhyan E.G.; Zvonkov E.E.; Parovichnikova E.N.; Savchenko V.G.

doi:https://doi.org/10.17116/terarkh20168874-14

Chernova N.G.

FGBU "Gematologicheskiĭ nauchnyĭ tsentr" Minzdrava Rossii, Moskva

Dzhulakian U.L.

FGBU "Gematologicheskiĭ nauchnyĭ tsentr" Minzdrava Rossii, Moskva

Vinogradova Yu.E.

I.M. Sechenov First Moscow State Medical University, Ministry of Health of Russia, Moscow, Russia

Sidorova Yu.V.

Department of Surgery, Russian Medical Academy of Continuing Vocational Education, Health Ministry of the Russian Federation, Moscow, Russia

Ryzhikova N.V.

National Research Center for Hematology, Ministry of Health of Russia, Moscow, Russia

Korzhova S.M.

National Research Center for Hematology, Ministry of Health of Russia, Moscow, Russia

Sinitsyna M.N.

FGBU "Gematologicheskiĭ nauchnyĭ tsent" Minzdrava Rossii, Moskva

Tikhomirov D.S.

FGBU "Gematologicheskiĭ nauchnyĭ tsentr" Minzdrava Rossii, Moskva

Naumova E.V.

Russian Medical Academy of Postgraduate Education, Ministry of Health of Russia, Moscow, Russia

Obukhova T.N.

FGBU "Gematologicheskiĭ nauchnyĭ tsentr" Minzdrava Rossii, Moskva

Dvirnyk V.N.

National research center for hematology, Ministry of Health of Russia, Moscow, Russia

Sudarikov A.B.

Gematologicheskiĭ nauchnyĭ tsentr Minzdrava Rossii, Moskva

Kovrigina A.M.

Nauchno-klinicheskoe otdelenie gematologicheskoĭ khirurgii Gematologicheskogo nauchnogo tsentra Minzdrava RF, Moskva

Kravchenko S.K.

Gematologicheskiĭ nauchnyĭ tsentr Minzdrava Rossii

Melikjan A.L.

GBOU VPO «Pervyj MGMU im. I.M. Sechenova» Minzdrava Rossii, Moskva

Kuz'mina L.A.

Gematologicheskiĭ nauchnyĭ tsentr Minzdrava Rossii, Moskva

Gal'tseva I.V.

GNTs Minzdrava Rossii, Moskva

Smirnova S.Yu.

National Research Center for Hematology, Ministry of Health of Russia, Moscow, Russia

Gemdzhyan E.G.

National Research Center for Hematology, Moscow, Russia

Zvonkov E.E.

Gematologicheskiĭ nauchnyĭ tsentr Minzdrava Rossii, Moskva

Parovichnikova E.N.

Federal state budgetary institution 'Hematological Research Centre', Russian Ministry of Health, Moscow, Russia

Savchenko V.G.

National Research Center for Hematology, Moscow, Russia

Hepatosplenic T-cell lymphoma: The problems of diagnosis and treatment

Authors:

Chernova N.G., Dzhulakian U.L., Vinogradova Yu.E., Sidorova Yu.V., Ryzhikova N.V., Korzhova S.M., Sinitsyna M.N., Tikhomirov D.S., Naumova E.V., Obukhova T.N., Dvirnyk V.N., Sudarikov A.B., Kovrigina A.M., Kravchenko S.K., Melikjan A.L., Kuz'mina L.A., Gal'tseva I.V., Smirnova S.Yu., Gemdzhyan E.G., Zvonkov E.E., Parovichnikova E.N., Savchenko V.G.

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Journal: Therapeutic Archive. 2016;88(7): 4‑14

DOI: 10.17116/terarkh20168874-14

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To cite this article:

Chernova NG, Dzhulakian UL, Vinogradova YuE, et al. . Hepatosplenic T-cell lymphoma: The problems of diagnosis and treatment. Therapeutic Archive. 2016;88(7):4‑14. (In Russ.)
https://doi.org/10.17116/terarkh20168874-14

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Abstract:

In the past decade, a notable advance has been made in the understanding of the pathogenesis of NK/T-cell lymphomas; however, their diagnosis remains difficult because of their rarity and clinical and morphological variabilities. The paper generalizes the ten-year experience of the Hematology Research Center, Ministry of Health of Russia, in diagnosing and treating hepatosplenic T-cell lymphoma (HSTL), considers the problems of differential diagnosis with other hematological diseases occurring with similar clinical and laboratory symptoms, and lays down current approaches to the diagnosis and treatment of this condition. A clinician’s view of the problem of diagnosis and treatment of this disease is given. HSTL is shown to be a heterogeneous group of diseases differing in a T-cell receptor chain gene rearrangement, the clinical course of the disease, and overall survival (OS). According to our data, 3-year OS was 12%; the median survival was 26 months. Two-year OS for γδ and αβ HSTL was equal to 25 and 70%, respectively. The difference in OS for the variants of HSTL failed to reach statistical significance (because the sample might be insufficient).

Keywords:

hepatosplenic T-cell lymphoma

immunohistochemical examination

clonal rearrangements

molecular study

morphology

flow cytometry

cytogenetic testing

Authors:

Chernova N.G.

FGBU "Gematologicheskiĭ nauchnyĭ tsentr" Minzdrava Rossii, Moskva

Dzhulakian U.L.

FGBU "Gematologicheskiĭ nauchnyĭ tsentr" Minzdrava Rossii, Moskva

Vinogradova Yu.E.

I.M. Sechenov First Moscow State Medical University, Ministry of Health of Russia, Moscow, Russia

Sidorova Yu.V.

Department of Surgery, Russian Medical Academy of Continuing Vocational Education, Health Ministry of the Russian Federation, Moscow, Russia

Ryzhikova N.V.

National Research Center for Hematology, Ministry of Health of Russia, Moscow, Russia

Korzhova S.M.

National Research Center for Hematology, Ministry of Health of Russia, Moscow, Russia

Sinitsyna M.N.

FGBU "Gematologicheskiĭ nauchnyĭ tsent" Minzdrava Rossii, Moskva

Tikhomirov D.S.

FGBU "Gematologicheskiĭ nauchnyĭ tsentr" Minzdrava Rossii, Moskva

Naumova E.V.

Russian Medical Academy of Postgraduate Education, Ministry of Health of Russia, Moscow, Russia

Obukhova T.N.

FGBU "Gematologicheskiĭ nauchnyĭ tsentr" Minzdrava Rossii, Moskva

Dvirnyk V.N.

National research center for hematology, Ministry of Health of Russia, Moscow, Russia

Sudarikov A.B.

Gematologicheskiĭ nauchnyĭ tsentr Minzdrava Rossii, Moskva

Kovrigina A.M.

Nauchno-klinicheskoe otdelenie gematologicheskoĭ khirurgii Gematologicheskogo nauchnogo tsentra Minzdrava RF, Moskva

Kravchenko S.K.

Gematologicheskiĭ nauchnyĭ tsentr Minzdrava Rossii

Melikjan A.L.

GBOU VPO «Pervyj MGMU im. I.M. Sechenova» Minzdrava Rossii, Moskva

Kuz'mina L.A.

Gematologicheskiĭ nauchnyĭ tsentr Minzdrava Rossii, Moskva

Gal'tseva I.V.

GNTs Minzdrava Rossii, Moskva

Smirnova S.Yu.

National Research Center for Hematology, Ministry of Health of Russia, Moscow, Russia

Gemdzhyan E.G.

National Research Center for Hematology, Moscow, Russia

Zvonkov E.E.

Gematologicheskiĭ nauchnyĭ tsentr Minzdrava Rossii, Moskva

Parovichnikova E.N.

Federal state budgetary institution 'Hematological Research Centre', Russian Ministry of Health, Moscow, Russia

Savchenko V.G.

National Research Center for Hematology, Moscow, Russia

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References:

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