Preprint Case Report Version 1 Preserved in Portico This version is not peer-reviewed

Epstein-Barr Virus-negative Diffuse Large B Cell Lymphoma in a X-linked Severe Combined Immunodeficiency Patient: A Very Rare Condition

Dina Rochate
* ORCID logo ,
Sara Duarte
,
Rita S. Gomes
,
Rita Pinho Peixeiro
,
João Vaz Silva
,
Ângelo Rodrigues
,
Luís Leite
,
Rosa Branca
,
Sónia Lemos
ORCID logo ,
Joana Guimarães
,
Susana Roncon
,
Ana Maia Ferreira
,
Carlos Pinho Vaz
Version 1 : Received: 15 December 2023 / Approved: 18 December 2023 / Online: 18 December 2023 (13:00:33 CET)

How to cite: Rochate, D.; Duarte, S.; Gomes, R.S.; Peixeiro, R.P.; Silva, J.V.; Rodrigues, Â.; Leite, L.; Branca, R.; Lemos, S.; Guimarães, J.; Roncon, S.; Ferreira, A.M.; Vaz, C.P. Epstein-Barr Virus-negative Diffuse Large B Cell Lymphoma in a X-linked Severe Combined Immunodeficiency Patient: A Very Rare Condition. Preprints 2023, 2023121230. https://doi.org/10.20944/preprints202312.1230.v1 Rochate, D.; Duarte, S.; Gomes, R.S.; Peixeiro, R.P.; Silva, J.V.; Rodrigues, Â.; Leite, L.; Branca, R.; Lemos, S.; Guimarães, J.; Roncon, S.; Ferreira, A.M.; Vaz, C.P. Epstein-Barr Virus-negative Diffuse Large B Cell Lymphoma in a X-linked Severe Combined Immunodeficiency Patient: A Very Rare Condition. Preprints 2023, 2023121230. https://doi.org/10.20944/preprints202312.1230.v1

Abstract

Severe combined immunodeficiency disorders (SCID) are a genetically heterogeneous group of inherited defects characterized by severe abnormalities of immune system development and function that lead to a wide spectrum in clinical manifestations. A subgroup of patients presents a disabling and life-threatening clinical course. In these cases, allogeneic hematopoietic stem cell transplant provides a curative approach. The occurrence of SCID-associated lymphoproliferative disorders is rare and occur mostly in adenosine deaminase-deficient severe combined immunodeficiency disorders, after allogeneic hematopoietic stem cell transplant. Epstein Barr virus infection is present in the majority of individuals with primary immunodeficiency developing lymphoma, revealing compromised anti-tumour surveillance of virally transformed cells. Herein, we report a rare case of diffuse large B cell lymphoma occurring in an Epstein Barr virus negative, non-transplanted X-linked severe combined immunodeficiency 4-months-old infant, successfully treated with immunotherapy and allogeneic stem cell transplantation. This case suggests that Epstein Barr virus -independent mechanism of neoplastic transformation must take action in severe combined immunodeficiency associated lymphoma and unveils the curative potential of donor T cells after allogeneic transplantation.

Keywords

DLBCL; X-linked SCID; allogeneic stem cell transplantation; infant; lymphoma

Subject

Medicine and Pharmacology, Transplantation

Copyright: This is an open access article distributed under the Creative Commons Attribution License which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Download PDF

Comments (0)

We encourage comments and feedback from a broad range of readers. See criteria for comments and our Diversity statement.

Leave a public comment
Send a private comment to the author(s)
* All users must log in before leaving a comment
Views 0
Downloads 0
Comments 0
Metrics 0
Get PDF Cite Share 0
Bookmark BibSonomy Mendeley Reddit Delicious
×
Alerts
Notify me about updates to this article or when a peer-reviewed version is published.
We use cookies on our website to ensure you get the best experience.
Read more about our cookies here.