lymphoproliferative disease

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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Autoimmune lymphoproliferative syndrome: literature review and case report

RARE AND COMPLEX HEMATOLOGICAL SYNDROMES , , , , , , ,

I.B. Kovynev1, T.I. Pospelova1, A.V. Mishenin2, I.N. Nechunaeva2, and R.V. Tarnovsky1

1 Novosibirsk State Medical University, Novosibirsk, Russian Federation

2 Municipal Clinical Hospital #2, Novosibirsk, Russian Federation

ABSTRACT

The article presents the literature review and the rare case of autoimmune lymphoproliferative syndrome (ALPS). The disease is associated with blocked apoptosis of immune cells caused by mutations in Fas-protein genes. The clinical course is characterized by progressive generalized lymphadenopathy with the picture of reactive hyperplasia in lymph nodes biopsy specimens and immune 1- to 3-lineage cytopenia(s). Potential of transformation into lymphoma or systemic lupus erythematosus (SLE) and current treatment approaches are discussed

Keywords: autoimmune lymphoproliferative syndrome, cytopenias, autoimmune hemolytic anemia, autoimmune neutropenia, lymphadenopathy, lymphoproliferative disease, immune thrombocytopenic purpura, lymphoma

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