Disseminated Histoplasma capsulatum in a Patient with Hyper IgM Immunodeficiency

PEDIATRICS ◽  
1994 ◽  
Vol 94 (2) ◽  
pp. 234-236
Author(s):  
Robert W. Hostoffer ◽  
Melvin Berger ◽  
Holly T. Clark ◽  
John R. Schreiber
Keyword(s):  
T Cell ◽  
Histoplasma Capsulatum ◽  
Pneumocystis Carinii ◽  
Immunoglobulin A ◽  
Serum Levels ◽  
Immunoglobulin M ◽  
Type I ◽  
Cell Class ◽  
Immunologic Abnormality ◽  
Hyper Igm

Immunodeficiency with hyper immunoglobulin M (IgM) (dysgammaglobulinemia type I) was first described in 1961.1 It is defined by low or absent serum levels of immunoglobulin G (IgG) and immunoglobulin A (IgA) but elevated or normal levels of IgM and immunoglobulin D.2 An intrinsic B cell class switching defect had been implicated as the pathophysiology.3,4 There is also strong evidence to suggest that a T cell defect, the absence of a T cell ligand for the CD4O antigen on B cells may be the underlying immunologic abnormality.5,6 Additionally, there is clinical evidence that these patients develop infections common to individuals with T cell defects such as Pneumocystis carinii,7 cryptococal meningitis and sepsis, (unpublished personal experience) Cryptosporidium,8 and Giardia lamblia.9

Activated T cells enhance interferon-α production by plasmacytoid dendritic cells stimulated with RNA-containing immune complexes

2015 ◽  
Vol 75 (9) ◽  
pp. 1728-1734 ◽  
Author(s):  
Dag Leonard ◽  
Maija-Leena Eloranta ◽  
Niklas Hagberg ◽  
Olof Berggren ◽  
Karolina Tandre ◽  
...  
Keyword(s):  
T Cells ◽  
Dendritic Cells ◽  
T Cell ◽  
Immune Complexes ◽  
Serum Levels ◽  
Plasmacytoid Dendritic Cells ◽  
Interferon Α ◽  
Type I ◽  
Activated T Cells ◽  
Gm Csf

ObjectivesPatients with systemic lupus erythematosus (SLE) have an ongoing interferon-α (IFN-α) production by plasmacytoid dendritic cells (pDCs). We investigated whether T cells can promote IFN-α production by pDCs.MethodsHuman pDCs were stimulated with immune complexes (ICs) containing U1 small nuclear ribonucleic proteins particles and SLE-IgG (RNA-IC) in the presence of T cells or T cell supernatants. T cells were activated by anti-CD3/CD28 antibodies or in a mixed leucocyte reaction. IFN-α and other cytokines were determined in culture supernatants or patient sera with immunoassays. The effect of interleukin (IL) 3 and granulocyte-macrophage-colony-stimulating factor (GM-CSF) on pDCs was examined by the use of antibodies, and the expression of CD80/CD86 was determined using flow cytometry.ResultsActivated T cells and supernatants from activated T cells increased IFN-α production by >20-fold. The stimulatory effect of T cell supernatants was reduced after depletion of GM-CSF (81%) or by blocking the GM-CSF receptor (55%–81%). Supernatant from activated T cells, furthermore, increased the frequency of CD80 and CD86 expressing pDCs stimulated with RNA-IC from 6% to 35% (p<0.05) and from 10% to 26% (p<0.01), respectively. Activated SLE T cells enhanced IFN-α production to the same extent as T cells from healthy individuals and a subset of patients with SLE had increased serum levels of GM-CSF.ConclusionsActivated T cells enhance IFN-α production by RNA-IC stimulated pDCs via GM-CSF and induce pDC maturation. Given the increased serum levels of GM-CSF in a subset of patients with SLE, these findings suggest that activated T cells may upregulate type I IFN production in SLE.

scholarly journals Antigen-presenting ILC3 regulate T cell–dependent IgA responses to colonic mucosal bacteria

2019 ◽  
Vol 216 (4) ◽  
pp. 728-742 ◽  
Author(s):  
Felipe Melo-Gonzalez ◽  
Hana Kammoun ◽  
Elza Evren ◽  
Emma E. Dutton ◽  
Markella Papadopoulou ◽  
...  
Keyword(s):  
T Cell ◽  
B Cells ◽  
Antigen Presentation ◽  
Immunoglobulin A ◽  
Lymphoid Cells ◽  
Host Interactions ◽  
Commensal Microbiota ◽  
Follicular Helper ◽  
Cell Class ◽  
Antigen Presenting

Intestinal immune homeostasis is dependent upon tightly regulated and dynamic host interactions with the commensal microbiota. Immunoglobulin A (IgA) produced by mucosal B cells dictates the composition of commensal bacteria residing within the intestine. While emerging evidence suggests the majority of IgA is produced innately and may be polyreactive, mucosal-dwelling species can also elicit IgA via T cell–dependent mechanisms. However, the mechanisms that modulate the magnitude and quality of T cell–dependent IgA responses remain incompletely understood. Here we demonstrate that group 3 innate lymphoid cells (ILC3) regulate steady state interactions between T follicular helper cells (TfH) and B cells to limit mucosal IgA responses. ILC3 used conserved migratory cues to establish residence within the interfollicular regions of the intestinal draining lymph nodes, where they act to limit TfH responses and B cell class switching through antigen presentation. The absence of ILC3-intrinsic antigen presentation resulted in increased and selective IgA coating of bacteria residing within the colonic mucosa. Together these findings implicate lymph node resident, antigen-presenting ILC3 as a critical regulatory checkpoint in the generation of T cell–dependent colonic IgA and suggest ILC3 act to maintain tissue homeostasis and mutualism with the mucosal-dwelling commensal microbiota.

scholarly journals Enhanced B Cell Expansion, Survival, and Humoral Responses by Targeting Death Receptor 6

2002 ◽  
Vol 197 (1) ◽  
pp. 51-62 ◽  
Author(s):  
Clint S. Schmidt ◽  
Jinqi Liu ◽  
Tonghai Zhang ◽  
Ho Yeong Song ◽  
George Sandusky ◽  
...  
Keyword(s):  
T Cell ◽  
B Cells ◽  
B Cell ◽  
Cell Expansion ◽  
Death Receptor ◽  
Immunoglobulin M ◽  
Type I ◽  
Factor C

Targeted disruption of death receptor (DR)6 results in enhanced CD4+ T cell expansion and T helper cell type 2 differentiation after stimulation. Similar to T cells, DR6 is expressed on resting B cells but is down-regulated upon activation. We examined DR6−/− B cell responses both in vitro and in vivo. In vitro, DR6−/− B cells undergo increased proliferation in response to anti–immunoglobulin M, anti-CD40, and lipopolysaccharide. This hyperproliferative response was due, at least in part, to both increased cell division and reduced cell apoptosis when compared with wild-type B cells. Consistent with these observations, increased nuclear levels and activity of nuclear factor κB transcription factor, c-Rel, and elevated Bcl-xl expression were observed in DR6−/− B cells upon stimulation. In addition, DR6−/− B cells exhibited higher surface levels of CD86 upon activation and were more effective as antigen-presenting cells in an allogeneic T cell proliferation response. DR6−/− mice exhibited enhanced germinal center formation and increased titers of immunoglobulins to T-dependent as well as T-independent type I and II antigens. This is the first demonstration of a regulatory role of DR6 in the activation and function of B cells.

Decreased serum levels of immunoglobulin A, immunoglobulin M and immunoglobulin G in a patient with primary biliary cirrhosis: A case report

2013 ◽  
Vol 44 (10) ◽  
pp. E261-E266 ◽  
Author(s):  
Takumi Kawaguchi ◽  
Toshimitsu Tanaka ◽  
Michitoshi Hashiguchi ◽  
Hiroaki Miyoshi ◽  
Jun Akiba ◽  
...  
Keyword(s):  
Case Report ◽  
Primary Biliary Cirrhosis ◽  
Immunoglobulin G ◽  
Immunoglobulin A ◽  
Serum Levels ◽  
Immunoglobulin M ◽  
Biliary Cirrhosis

scholarly journals Relationship Between T-Cell Exosomes and Cellular Subsets in SLE According to Type I IFN-Signaling

2020 ◽  
Vol 7 ◽  
Author(s):  
Patricia López ◽  
Javier Rodríguez-Carrio ◽  
Luis Caminal-Montero ◽  
Ana Suárez
Keyword(s):  
Flow Cytometry ◽  
T Cells ◽  
T Cell ◽  
Lupus Erythematosus ◽  
Serum Levels ◽  
Type I ◽  
Type I Ifn ◽  
Activation Markers ◽  
Cytokines And Chemokines ◽  
Cytokine Milieu

Objective: To quantify the levels of circulating exosomes derived from T-cells and monocytes and their possible associations with leukocyte subpopulations and cytokine milieu in Systemic Lupus Erythematosus (SLE).Methods: Total circulating exosomes (CD9+-Ex) and those derived from T-cells (CD3+-Ex) and monocytes (CD14+-Ex) were quantified by flow cytometry in 82 SLE patients and 32 controls. Leukocyte subsets and serum cytokines were analyzed by flow cytometry or by immunoassays. IFN-score was evaluated by real time RT-PCR in whole blood samples from a subgroup of 73 patients and 24 controls.Results: Activation markers (IFNR1 and BLyS) on monocytes, neutrophils and B-cells correlated inversely with circulating exosomes (CD9+-Ex, CD3+-Ex, and CD14+-Ex) in controls but directly with CD3+-Ex in patients (all p &lt; 0.05). Although CD9+-Ex were increased in SLE, no differences were found in CD3+-Ex, supporting that exosome content accounts for this opposite role. Interestingly, CD4+CD28null cells correlated with CD3+-Ex in patients and controls, and displayed similar associations with leukocyte subsets in both groups. Additionally, CD3+-Ex correlated in patients with the expression of CD25 in CD4+CD28null cells. Furthermore, the activated status of this senescent subset was related to IFNα serum levels in controls and to IFN-score in SLE patients. Finally, patients presenting high IFN-score, in addition to elevated CD25+CD28null cells associated with the activation of myeloid cells, displayed higher levels of inflammatory cytokines and chemokines.Conclusion: Our results support a relationship between T-cell exosomes and cellular subsets in SLE according to type I IFN-signaling, which could amplify chronic immune activation and excessive cytokine/chemokine response.

Elevated serum levels of soluble E- and L-selectin in patients with human T-cell lymphotropic virus type I-associated myelopathy

1998 ◽  
Vol 155 (1) ◽  
pp. 76-79 ◽  
Author(s):  
Akira Tsujino ◽  
Tatsufumi Nakamura ◽  
Takafumi Furuya ◽  
Hirofumi Goto ◽  
Yoshihiro Nishiura ◽  
...  
Keyword(s):  
T Cell ◽  
Virus Type ◽  
Elevated Serum ◽  
Serum Levels ◽  
Type I ◽  
Human T Cell

SELECTIVE IMMUNOGLOBULIN DEFICIENCY ASSOCIATED WITH THYMIC ALYMPHOPLASIA

PEDIATRICS ◽  
1967 ◽  
Vol 39 (4) ◽  
pp. 506-515
Author(s):  
Rebecca H. Buckley ◽  
William D. Bradford ◽  
Suzanne R. Butcher
Keyword(s):  
Lymphoid Tissue ◽  
Fluorescent Antibody ◽  
Pneumocystis Carinii ◽  
Serum Levels ◽  
Postmortem Examination ◽  
Immunoglobulin M ◽  
Moderate Number ◽  
Peripheral Lymphoid Tissue ◽  
Immunoglobulin Deficiency ◽  
Rare Cells

Serum immunoglobulin, fluorescent antibody and pathological studies were performed on tissues from a 9-month-old infant who died of Pneumocystis carinii pneumonia. The serum levels of IgG and IgA were found to be greatly diminished, whereas IgM and IgD were present in normal amounts. In addition, a moderate number of immunoglobulin-M-containing-cells were demonstrated in the spleen by fluorescence microscopy. Rare cells were seen which contained IgA or IgG. The thymus was poorly developed, contained no Hassall's corpuscles and few lymphocytes. Peripheral lymphoid tissue was scant; only two lymph nodes and no tonsils were found at postmortem examination. The largest peripheral aggregation of small lymphocytes appeared in the appendix. During life the child manifested varying degrees of lymphopenia.

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