Massive expansion of maternal T cells in response to EBV infection in a patient with SCID-Xl

Touzot, Fabien; Dal-Cortivo, Liliane; Verkarre, Virginie; Lim, Annick; Crucis-Armengaud, Anne; Moshous, Despina; Héritier, Sébastien; Frange, Pierre; Kaltenbach, Sophie; Blanche, Stéphane; Pïcard, Capucine; Hacein‐Bey‐Abina, Salima; Cavazzana, Marina; Fischer, Alain

doi:10.1182/blood-2012-04-426833

Cited by 22 publications

(20 citation statements)

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“…In some rare cases, these cells either conferred immunity and protection against infection or they caused allograft rejection and immune cytopenias . A massive expansion of maternal T cells in response to Epstein–Barr virus infection in a patient with X‐linked SCID has been also reported . The first report of long‐term co‐existence of autologous T cells and high levels of engraftment of haploidentical maternal T cells was published recently .…”

Section: Discussionmentioning

confidence: 99%

Co-existence of clonal expanded autologous and transplacental-acquired maternal T cells in recombination activating gene-deficient severe combined immunodeficiency

Lev

Simon

Ben‐Ari

et al. 2014

Clinical and Experimental Immunology

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Section: Discussionmentioning

confidence: 99%

Co-existence of clonal expanded autologous and transplacental-acquired maternal T cells in recombination activating gene-deficient severe combined immunodeficiency

Lev

Simon

Ben‐Ari

et al. 2014

Clinical and Experimental Immunology

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“…We extracted genomic DNA from the patient's purified circulating B lymphocytes and performed microsatellite genotyping to test whether this immunohematologic restoration could originate from maternal cells, as observed in other situations. 18 This analysis indicated that the circulating B cells were of the patient's own origin and not from his mother (see Fig E3 in this article's Online Repository at www. jacionline.org).…”

Section: The Patient Experienced Spontaneous In Vivo Genetic Reversionmentioning

confidence: 97%

An in vivo genetic reversion highlights the crucial role of Myb-Like, SWIRM, and MPN domains 1 (MYSM1) in human hematopoiesis and lymphocyte differentiation

Guen

Touzot

André-Schmutz

et al. 2015

Journal of Allergy and Clinical Immunology

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“…The proportion of patients that develop an EBV‐driven B‐LPD varies according to the type of genetic defect and the age at diagnosis of the PID. In PIDs in which T cell development is severely impaired (such as SCID with B cells but no T cells (SCID T‐B+), resulting from mutations in IL2RG , JAK3 , IL7RA or CD3 subunits) or combined immunodeficiency (CID) caused by mutations in ZAP70 (characterized by the almost total absence of CD8 T cells), few cases of severe EBV infections have been documented . Patients who carry hypomorphic mutations in genes causing SCID T‐B‐ (such as RAG1/2 , LIG4 and DCLRE1C ) may also experience severe B‐cell lymphoproliferative disorders in which B‐cell development is preserved to some extent .…”

Section: Immunodeficiencies Causing Ebv‐driven B‐lymphoproliferative mentioning

confidence: 99%

Signaling pathways involved in the T‐cell‐mediated immunity against Epstein‐Barr virus: Lessons from genetic diseases

Latour

Fischer

2019

Immunological Reviews

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113

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Primary immunodeficiencies (PIDs) provide researchers with unique models to understand in vivo immune responses in general and immunity to infections in particular. In humans, impaired immune control of Epstein-Barr virus (EBV) infection is associated with the occurrence of several different immunopathologic conditions; these include non-malignant and malignant B-cell lymphoproliferative disorders, hemophagocytic lymphohistiocytosis (HLH), a severe inflammatory condition, and a chronic acute EBV infection of T cells. Studies of PIDs associated with a predisposition to develop severe, chronic EBV infections have led to the identification of key components of immunity to EBV -notably the central role of T-cell expansion and its regulation in the pathophysiology of EBV-associated diseases. On one hand, the defective expansion of EBV-specific CD8 T cells results from mutations in genes involved in T-cell activation (such as RASGRP1, MAGT1, and ITK), DNA metabolism (CTPS1) or co-stimulatory pathways (CD70, CD27, and TNFSFR9 (also known as CD137/4-1BB)) leads to impaired elimination of proliferating EBV-infected B cells and the occurrence of lymphoma. On the other hand, protracted T-cell expansion and activation after the defective killing of EBV-infected B cells is caused by genetic defects in the components of the lytic granule exocytosis pathway or in the small adapter protein SH2D1A (also known as SAP), a key activator of T-and NK cell-cytotoxicity. In this setting, the persistence of EBV-infected cells results in HLH, a condition characterized by unleashed T-cell and macrophage activation. Moreover, genetic defects causing selective vulnerability to EBV infection have highlighted the role of co-receptor molecules (CD27, CD137, and SLAM-R) selectively involved in immune responses against infected B cells via specific T-B cell interactions. K E Y W O R D S B-cell lymphoproliferation, cell-cytotoxicity, Epstein-Barr virus, hemophagocytic lymphohistiocytosis, primary immunodeficiency, T-cell proliferation | IMMUNE RE S P ON S E S TO EBV INFEC TI ONEpstein-Barr virus (EBV, also known as human herpesvirus 4) is a gamma herpes virus that only infects primates (primarily humans). 1,2 It is an encapsidated, double-stranded DNA virus with more than 100 genes. It is pandemic, since the great majority of human beings (>90%) have been infected by EBV. The main cell target is B lymphocytes that express CD21 -the membrane receptor for EBV entry through its 350 kDa major envelope glycoprotein. In immunocompetent adolescents and adults, EBV infection causes infectious mononucleosis (IM). Immunologically speaking, IM is characterized by a This article is part of a series of reviews covering Signaling and Signal Diversification in Immune

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Massive expansion of maternal T cells in response to EBV infection in a patient with SCID-Xl

Cited by 22 publications

References 10 publications

Co-existence of clonal expanded autologous and transplacental-acquired maternal T cells in recombination activating gene-deficient severe combined immunodeficiency

Co-existence of clonal expanded autologous and transplacental-acquired maternal T cells in recombination activating gene-deficient severe combined immunodeficiency

An in vivo genetic reversion highlights the crucial role of Myb-Like, SWIRM, and MPN domains 1 (MYSM1) in human hematopoiesis and lymphocyte differentiation

Signaling pathways involved in the T‐cell‐mediated immunity against Epstein‐Barr virus: Lessons from genetic diseases

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