Increased lymphocyte viability after non-stimulated peripheral blood mononuclear cell (PBMC) culture in patients with X-linked lymphoproliferative disease (XLP)

Belmonte, Liliana; Parodi, Cecilia; Bastón, Mariela; Coraglia, Ana; Felippo, Marta; Baré, Patricia; Malbrán, Alejandro; Ruibal-Ares, B; Bracco, María de E. de M.

doi:10.1016/j.clim.2009.05.015

Cited by 4 publications

(2 citation statements)

References 20 publications

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“…33 EBV-infected B cells that persist in XLP1 patients probably serve as persistent weak antigen-presenting cells for CD8 ϩ T cells as well, providing chronic T-cell restimulation that fails to trigger RICD. This newly recognized proapoptotic function for SAP in RICD and other programmed cell death pathways (ie, p53-response to DNA damage) is consistent with previous observations, 38 including the in vitro persistence of interferon-␥ high CD8 T cells from XLP patients in unstimulated cultures, an observation reminiscent of the descriptions of Bar et al in 1974. 3 …”

Section: Immunopathology Of Xlp1/sap Deficiencysupporting

confidence: 77%

X-linked lymphoproliferative syndromes: brothers or distant cousins?

Filipovich

Zhang

Snow

et al. 2010

Blood

151

168

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X-linked lymphoproliferative disease (XLP1), described in the mid-1970s and molecularly defined in 1998, and XLP2, reported in 2006, are prematurely lethal genetic immunodeficiencies that share susceptibility to overwhelming inflammatory responses to certain infectious triggers. Signaling lymphocytic activation molecule-associated protein (SAP; encoded by SH2D1A) is mutated in XLP1, and X-linked inhibitor of apoptosis (XIAP; encoded by BIRC4) is mutated in XLP2. XLP1 is a disease with multiple and variable clinical consequences, including fatal hemophagocytic lymphohistiocytosis (HLH) triggered predominantly by Epstein-Barr virus, lymphomas, antibody deficiency, and rarer consequences of immune dysregulation. To date, XLP2 has been found to cause HLH with and without exposure to Epstein-Barr virus, and HLH is commonly recurrent in these patients. For both forms of XLP, the only curative therapy at present is allogeneic hematopoietic cell transplantation. Beyond their common X-linked locus and their requirement for normal immune responses to certain viral infections, SAP and XIAP demonstrate no obvious structural or functional similarity, are not coordinately regulated with respect to their expression, and do not appear to directly interact. In this review, we describe the genetic, clinical, and immunopathologic features of these 2 disorders and discuss current diagnostic and therapeutic strategies.

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Section: Immunopathology Of Xlp1/sap Deficiencysupporting

confidence: 77%

X-linked lymphoproliferative syndromes: brothers or distant cousins?

Filipovich

Zhang

Snow

et al. 2010

Blood

151

168

View full text Add to dashboard Cite

show abstract

“…However, somatically mutated IgM + CD27 + , but not Ig-subtype switched B cells could be found in XLP despite the absence of germinal centres in secondary lymphoid organs (Coraglia et al, 2010). Likewise, after prolonged culture of peripheral blood mononuclear cells (PBMC), the percentage of residual surviving B lymphocytes having a non-switched memory phenotype (IgM + IgD + CD27 + ) was higher in XLP patients than normal controls (Belmonte et al, 2009). We studied the long-term (>10 years) evolution of the EBV viral load (EBV VL) and B-lymphocyte profile in two surviving hypogammaglobulinaemic siblings of an established XLP family (Malbran et al, 2001).…”

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confidence: 99%

Progressive reduction of circulating B lymphocytes in patients with X‐linked lymphoproliferative disease (XLP)

et al. 2016

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In vitro lymphocyte stimulation by recombinant hepatitis B surface antigen: A tool to detect the persistence of cellular immunity after vaccination

Livramento

Sampaio

Schultz

et al. 2013

Journal of Virological Methods

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The study of the human immune response to hepatitis B virus (HBV) has been hampered by the lack of an adequate model to evaluate the hepatitis B surface antigen (HBsAg) specific cell response. Thus, this study was conducted to perform an in vitro analysis of the antigenic properties of recombinant HBsAg and demonstrate the influence of variables such as culture time, antigen concentration and cell density on lymphocyte proliferation. Peripheral blood mononuclear cells (PBMCs) were isolated from the venous blood of vaccinated individuals, and in vitro cellular immune responses were evaluated using an HBsAg-specific proliferation assay. Lymphoproliferative responses were detected in culture systems, despite the lack of serum antibodies. Optimal results were obtained when lymphocytes were stimulated at a seeding density of 4×10(6) cells/mL, with 50 ng/mL of recombinant HBsAg protein vaccine for 3 days. Data from the present study may contribute to the development of an adequate system to evaluate the cellular immune responses to HBsAg in vaccine recipients.

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Increased lymphocyte viability after non-stimulated peripheral blood mononuclear cell (PBMC) culture in patients with X-linked lymphoproliferative disease (XLP)

Cited by 4 publications

References 20 publications

X-linked lymphoproliferative syndromes: brothers or distant cousins?

X-linked lymphoproliferative syndromes: brothers or distant cousins?

Progressive reduction of circulating B lymphocytes in patients with X‐linked lymphoproliferative disease (XLP)

In vitro lymphocyte stimulation by recombinant hepatitis B surface antigen: A tool to detect the persistence of cellular immunity after vaccination

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