Systemic Т-Cell Lymphoproliferative Disease Associated with Epstein-Barr Virus: A Literature Review and a Case Report

AUTOIMMUNE THROMBOCYTOPENIA , ,

EA Shalamova, AM Kovrigina, IA Shupletsova, EE Nikulina, VD Latyshev, NV Tsvetaeva

National Research Center for Hematology, 4 Novyi Zykovskii pr-d, Moscow, Russian Federation, 125167

For correspondence: Alla Mikhailovna Kovrigina, PhD in Biology, 4 Novyi Zykovskii pr-d, Moscow, Russian Federation, 125167; e-mail: kovrigina.alla@gmail.com

For citation: Shalamova EA, Kovrigina AM, Shupletsova IA, et al. Systemic Т-Cell Lymphoproliferative Disease Associated with Epstein-Barr Virus: A Literature Review and a Case Report. Clinical oncohematology. 2021;14(4):477–87. (In Russ).

DOI: 10.21320/2500-2139-2021-14-4-477-487

ABSTRACT

Epstein-Barr virus (EBV) is ubiquitous, being identified in 90–95 % of adults. Its reactivation in immunodeficiency conditions often leads to clonal transformation of В-lymphocytes and development of В-cell lymphoproliferative diseases (LPD) and В-cell lymphomas. At the same time, in the countries of North-East and East Asia, as well as Latin America, non-immunocompromised patients sometimes demonstrate the development of EBV-associated Т-cell lymphoproliferative diseases. The present paper reports a rare case of EBV-associated systemic T-LPD with lymphadenopathy, splenomegaly as well as acute autoimmune hemolytic anemia in a man of Caucasian race. Complex analysis of anamnestic, pathomorphological, and laboratory data allowed to distinguish this disease from Т-cell lymphoma and choose the appropriate patient management strategy.

Keywords: lymphoproliferative disease, Epstein-Barr virus, EBV+ T-LPD, diagnosis, pathomorphology.

Received: May 30, 2021

Accepted: September 2, 2021

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Статистика Plumx английский

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