Two clonally distinct B-cell lymphomas reveal the diagnosis of XLP1 in a male child and his asymptomatic male relatives: Case report and review of the literature Review

  

Authors:	Iglesias Cardenas, F.; Agarwal, A. M.; Vagher, J.; Maese, L.; Fluchel, M.; Afify, Z.
Review Title:	Two clonally distinct B-cell lymphomas reveal the diagnosis of XLP1 in a male child and his asymptomatic male relatives: Case report and review of the literature
Abstract:	X-linked lymphoproliferative disease type 1 (XLP1) is a primary immunodeficiency disorder caused by pathogenic variants in the SH2D1A gene (SH2 domain containing protein 1A). Patients with XLP1 may present acutely with fulminant infectious mononucleosis, hemophagocytic lymphohistiocytosis, and/or B-cell non-Hodgkin lymphoma (B-NHL). We report a boy who developed 2 clonally distinct B-NHL 4 years apart and was found to have previously unrecognized XLP1. The report highlights the importance of clonal analysis and XLP1 testing in males with presumed late recurrences of B-NHL, and the role of allogeneic stem cell transplant (allo-SCT) in XLP1 patients and their affected male relatives. Copyright © 2021 Wolters Kluwer Health, Inc. All rights reserved.
Journal Title:	Journal of Pediatric Hematology/Oncology
Volume:	43
Issue:	8
ISSN:	1077-4114
Publisher:	Lippincott Williams & Wilkins
Date Published:	2021-11-01
Start Page:	e1210
End Page:	e1213
Language:	English
DOI:	10.1097/MPH.0000000000002049
PUBMED:	33448720
PROVIDER:	scopus
DOI/URL:	https://www.scopus.com/inward/record.uri?eid=2-s2.0-85120522496&doi=10.1097%2fMPH.0000000000002049&partnerID=40&md5=c7d734a544487f54c9e736f1ff6e8c0f
Notes:	Article -- Export Date: 3 January 2022 -- Source: Scopus

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