Lymphoma as a rare complication of severe combined immunodeficiency

D. A. Venyov; Венёв Д. А.; E. V. Deripapa; Дерипапа Е. В.; A. A. Roppelt; Роппельт А. А.; A. L. Laberko; Лаберко А. Л.; D. S. Abramov; Абрамов Д. С.; T. V. Varalamova; Варламова Т. В.; A. A. Mukhina; Мухина А. А.; D. N. Balashov; Балашов Д. Н.; A. Yu. Shcherbina; Щербина А. Ю.

doi:10.24287/1726-1708-2019-18-1-104-111

Lymphoma as a rare complication of severe combined immunodeficiency

Authors: Venyov D.A.¹, Deripapa E.V.¹, Roppelt A.A.¹, Laberko A.L.¹, Abramov D.S.¹, Varalamova T.V.¹, Mukhina A.A.¹, Balashov D.N.¹, Shcherbina A.Y.¹
Affiliations:
1. Dmitriy Rogachev National Medical Research Center of Pediatric Hematology, Oncology, Immunology Ministry of Healthcare of Russian Federation
Issue: Vol 18, No 1 (2019)
Pages: 104-111
Section: SCHOOL OF IMMUNOLOGY – EXPERT OPINION
Submitted: 21.04.2019
Accepted: 21.04.2019
Published: 21.04.2019
URL: https://hemoncim.com/jour/article/view/232
DOI: https://doi.org/10.24287/1726-1708-2019-18-1-104-111
ID: 232

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Abstract

Primary immunodeficiencies (PID) are rare genetically determined disorders of immune system. PID are most often manifested by infectious and autoimmune complications. However, as the life expectancy of patients with PID has increased as a result of improved therapy, the increased risk of developing malignancies, primarily lymphomas, became more prominent. The incidence of malignancies in patients with PID ranges from 4 to 60%. The role of oncoviruses is important in the pathogenesis of lymphomas in PID, but there are also non-infectious causes. Lymphomas in severe combined immunodeficiency (SCID) are extremely rare, and are usually represented by post-transplant lymphoproliferative diseases (PTLD). The development of true lymphomas in SCID is an extremely rare event. We describe two clinical cases of lymphomas in children with X-linked SCID, with successful non-protocol therapy of lymphoma and subsequent transplantation of hematopoietic stem cells. Parents gave their consent to use information about the child in the article.