Epstein–Barr virus-associated smooth muscle tumors in patients with primary immunodeficiencies

Z. А. Nesterenko; Нестеренко З. А.; A. А. Roppelt; Роппельт А. А.; Yu. A. Rodina; Родина Ю. А.; A. A. Moiseeva; Моисеева А. А.; E. V. Deripapa; Дерипапа Е. В.; A. L. Kozlova; Козлова А. Л.; D. S. Abramov; Абрамов Д. С.; D. Yu. Kachanov; Качанов Д. Ю.; M. V. Teleshova; Телешова М. В.; S. R. Talypov; Талыпов С. Р.; E. V. Raykina; Райкина Е. В.; I. V. Mersiyanova; Мерсиянова И. В.; V. V. Zakharova; Захарова В. В.; S. G. Mann; Манн С. Г.; G. V. Tereshchenko; Терещенко Г. В.; A. Yu. Shcherbina; Щербина А. Ю.

doi:10.24287/1726-1708-2020-19-4-165-177

Epstein–Barr virus-associated smooth muscle tumors in patients with primary immunodeficiencies

Authors: Nesterenko Z.А.¹, Roppelt A.А.¹, Rodina Y.A.¹, Moiseeva A.A.¹, Deripapa E.V.¹, Kozlova A.L.¹, Abramov D.S.¹, Kachanov D.Y.¹, Teleshova M.V.¹, Talypov S.R.¹, Raykina E.V.¹, Mersiyanova I.V.¹, Zakharova V.V.¹, Mann S.G.¹, Tereshchenko G.V.¹, Shcherbina A.Y.¹
Affiliations:
1. Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology of Ministry of Healthcare of the Russian Federation
Issue: Vol 19, No 4 (2020)
Pages: 165-177
Section: CLINICAL OBSERVATIONS
Submitted: 21.12.2020
Accepted: 21.12.2020
Published: 08.07.2025
URL: https://hemoncim.com/jour/article/view/433
DOI: https://doi.org/10.24287/1726-1708-2020-19-4-165-177
ID: 433

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Abstract

Epstein–Barr virus-associated smooth muscle tumors (EBV-SMTs) are rare soft tissue neoplasms that typically occur in conditions of secondary immunodeficiency due to human immunodeficiency virus or immunosuppression after organ transplantation. Beyond that, EBV-SMTs occur in patients with primary immunodeficiencies (PIDs). EBV-SMTs pathogenesis is still unclear but it was shown that the key mechanism of the tumor development is T- and NK-cell defect. Treatment strategy depends not only on tumor localization and resectability, but also on immunodeficiency etiology and its correction possibility. Here we report literature review and two patients with combined PIDs (CARMIL2- and ATM-genes deficiency) who developed EBV-SMT. In each case, the parents gave consent to the use of their child's data, including photographs, for research purposes and in publications.

Keywords

Epstein–Barr virus, smooth muscle tumors, primary immunodeficiencies

About the authors

Z. А. Nesterenko

Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology of Ministry of Healthcare of the Russian Federation

ORCID iD: 0000-0003-4427-054X
Moscow Russian Federation

A. А. Roppelt

Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology of Ministry of Healthcare of the Russian Federation

Author for correspondence.
Email: roppelt_anna@mail.ru
ORCID iD: 0000-0001-5132-1267

Anna A. Roppelt, doctor in the Department of Immunology

1 Samory Mashela St., Moscow 117997

Russian Federation

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