The Natural History of X-Linked Lymphoproliferative Disease (XLP1): Lessons from a Long-Term Survivor
Joint Authors
Jiang, Yike
Firan, Mihail
Nandiwada, Sarada L.
Reyes, Anaid
Marsh, Rebecca A.
Vogel, Tiphanie P.
Hajjar, Joud
Source
Issue
Vol. 2020, Issue 2020 (31 Dec. 2020), pp.1-9, 9 p.
Publisher
Hindawi Publishing Corporation
Publication Date
2020-08-27
Country of Publication
Egypt
No. of Pages
9
Main Subjects
Abstract EN
X-linked lymphoproliferative disease (XLP1) is a rare primary immunodeficiency characterized by EBV-triggered immune dysregulation, lymphoproliferation, dysgammaglobulinemia, and lymphoma.
Early childhood mortality from overwhelming inflammation is expected in most patients.
The only curative therapy is hematopoietic stem cell transplant (HSCT); however, whether to perform HSCT on an asymptomatic patient remains debatable.
This uncertainty arises because the natural history of XLP1 patients without transplantation is not clear.
In this case report, we present the natural history of XLP1 in a 43-year-old male patient who did not receive HSCT.
We also review the literature on untransplanted XLP1 patients who lived into mid-adulthood.
Despite surviving childhood presentations that are typically fatal, we found that these rare patients remain susceptible to manifestations of XLP1 decades later.
American Psychological Association (APA)
Jiang, Yike& Firan, Mihail& Nandiwada, Sarada L.& Reyes, Anaid& Marsh, Rebecca A.& Vogel, Tiphanie P.…[et al.]. 2020. The Natural History of X-Linked Lymphoproliferative Disease (XLP1): Lessons from a Long-Term Survivor. Case Reports in Immunology،Vol. 2020, no. 2020, pp.1-9.
https://search.emarefa.net/detail/BIM-1148013
Modern Language Association (MLA)
Jiang, Yike…[et al.]. The Natural History of X-Linked Lymphoproliferative Disease (XLP1): Lessons from a Long-Term Survivor. Case Reports in Immunology No. 2020 (2020), pp.1-9.
https://search.emarefa.net/detail/BIM-1148013
American Medical Association (AMA)
Jiang, Yike& Firan, Mihail& Nandiwada, Sarada L.& Reyes, Anaid& Marsh, Rebecca A.& Vogel, Tiphanie P.…[et al.]. The Natural History of X-Linked Lymphoproliferative Disease (XLP1): Lessons from a Long-Term Survivor. Case Reports in Immunology. 2020. Vol. 2020, no. 2020, pp.1-9.
https://search.emarefa.net/detail/BIM-1148013
Data Type
Journal Articles
Language
English
Notes
Includes bibliographical references
Record ID
BIM-1148013