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Category: Immunology
Antibody Deficiencies, Page 1 of 2
< Previous page | Next page > /docserver/preview/fulltext/10.1128/9781555818722/9781555818715_CH76-1.gif /docserver/preview/fulltext/10.1128/9781555818722/9781555818715_CH76-2.gifAbstract:
Antibody deficiency can be defined as a condition characterized by a reduction in serum immunoglobulin concentrations below the fifth centile for age. Antibody deficiency may affect all classes of immunoglobulins or may be confined to a single isotype.
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Investigation of possible antibody deficiency. This picture shows laboratory investigations in patients suspected of antibody deficiency.
Investigation of possible antibody deficiency. This picture shows laboratory investigations in patients suspected of antibody deficiency.
Extended B-cell immunophenotyping. The FACS plots demonstrate the gating strategy for B-cell immunophenotyping. Lymphocytes are gated based on forward (FSC) versus side scatter (SSC) (panel 1). The lymphocyte population is then used to gate B cells (CD19 versus side scatter) (panel 2). Using quadrants, naive B cells are defined as IgD+/CD27−, IgD+/CD27+ are non-switched memory B cells, and IgD−/CD27+ are switched memory B cells (panel 3). Transitional B cells are defined as IgM++CD38+ and IgM-CD38+ B cells and plasmablasts as IgM-CD38+ (panel 4). CD21low/CD38low B cells are indicated in panel 5. The presence of double-positive B cells greatly decreases the possibility of mutations in the AID gene, and they are used for back gating to confirm the IgM status of naive, switched, and non-switched memory B cells.
Extended B-cell immunophenotyping. The FACS plots demonstrate the gating strategy for B-cell immunophenotyping. Lymphocytes are gated based on forward (FSC) versus side scatter (SSC) (panel 1). The lymphocyte population is then used to gate B cells (CD19 versus side scatter) (panel 2). Using quadrants, naive B cells are defined as IgD+/CD27−, IgD+/CD27+ are non-switched memory B cells, and IgD−/CD27+ are switched memory B cells (panel 3). Transitional B cells are defined as IgM++CD38+ and IgM-CD38+ B cells and plasmablasts as IgM-CD38+ (panel 4). CD21low/CD38low B cells are indicated in panel 5. The presence of double-positive B cells greatly decreases the possibility of mutations in the AID gene, and they are used for back gating to confirm the IgM status of naive, switched, and non-switched memory B cells.
CD40L expression. Lymphocytes are defined by CD45 versus SSC. Prior to stimulation, there is limited CD25 and CD40L (CD154) expression (“Unstim” panels). Post stimulation in healthy individuals, there is an increase in both CD40L and CD25 expression (middle “Stim” panels). In patients with XHIM, although there is significant upregulation of CD25 (bottom right “Stim” panel), there is no increase in CD40L expression (top right “Stim” panel).
CD40L expression. Lymphocytes are defined by CD45 versus SSC. Prior to stimulation, there is limited CD25 and CD40L (CD154) expression (“Unstim” panels). Post stimulation in healthy individuals, there is an increase in both CD40L and CD25 expression (middle “Stim” panels). In patients with XHIM, although there is significant upregulation of CD25 (bottom right “Stim” panel), there is no increase in CD40L expression (top right “Stim” panel).
BTK analysis by FACs. Monocytes are gated by CD14+ versus SSC. Histograms showing isotype staining and BTK staining are then overlaid. Many hypomorphic mutations in BTK show a partial overlay as shown in the patient sample.
BTK analysis by FACs. Monocytes are gated by CD14+ versus SSC. Histograms showing isotype staining and BTK staining are then overlaid. Many hypomorphic mutations in BTK show a partial overlay as shown in the patient sample.
XIAP and SAP by FACs. Lymphocytes are defined based on FSC vs. SSC (panel 1). CD8+ T cells are then gated based on CD3+/CD8+ (panel 2). Isotype, XIAP (green), and SAP (pink) are analyzed as overlaid histograms (panel 3), where the XIAP shift about 1 log and the SAP about 2 logs. An XLP2 patient lacks XIAP (panel 4). An XLP1 patient lacks SAP (panel 5).
XIAP and SAP by FACs. Lymphocytes are defined based on FSC vs. SSC (panel 1). CD8+ T cells are then gated based on CD3+/CD8+ (panel 2). Isotype, XIAP (green), and SAP (pink) are analyzed as overlaid histograms (panel 3), where the XIAP shift about 1 log and the SAP about 2 logs. An XLP2 patient lacks XIAP (panel 4). An XLP1 patient lacks SAP (panel 5).
The main phenotypes of primary antibody deficiencies a
The main phenotypes of primary antibody deficiencies a
Inheritance of antibody deficiencies
Inheritance of antibody deficiencies