Glossary of HD - SGPSS 5 (B-Cell Deficiency)
Other Decks By This User
- What is X-Linked Agammaglobulinemia (XLA)?
- An X-linked defect in B-Cell development which prevents differentiation and proliferation of B-Cells, absence of plasma cells, absence of germinal centers, and very low levels of circulating immunoglobulins.
- Why would a patient with XLA present with persistent infection?
- Lack of Ab's lead to an inability to opsonize capsular antigens and suboptimal binding to C3b and Fc-gamma receptors on neutrophils resulting in ineffective phagocytosis.
- What kind of therapy is appropriate for XLA?
- IgG replacement therapy (passive immunity).
- What is Severe Combined ImmunoDeficiency (SCID)?
- Insufficient lymphocytes resulting in a broad inability to respond to infection early after birth.
- What cells will be markedly reduced in SCID?
- Neutrophils, platelets, lymphocytes due to an early lineage defect in hemopoietic stem cells in the bone marrow.
RBC's are not affected due to an early break in lineage.
- What would an electrophoresis of a patient with SCID look like?
- Reduced globulins.
- What would a FACS analysis (separate and measure different cell types) of SCID look like?
- Absence of all lymphocytes.
- What is Mixed Lymphocyte Testing and why is it useful?
- Test to find the best donor-recipient match. Mix each blood and find the least amount of proliferation (with one side radioactively suppressed).
- What is MHC Typing and what is it useful for?
- One parent gives a set of MHC's to the child, and it helps to determine positive matches for organ transplant. What else??
- What are the symptoms of a Plasma cell defect?
- High number of circulating lymphocytes, but low serum levels of IgG.
- What prevents B-Cell maturation in patients with a Plasma cell defect?
- No germinal centers for isotype switching and terminal differentiation.
- What is the difference between XLA and plasma cell defects?
- XLA would have no Ab's at all.
- Where might the possible defects be in the plasma cell defect?
- Defective B-cell differentiation, defective germinal centers, defective cytokines, defective B-cell to Th2 cell cross-talk.
- What are patients with plasma cell defects more susceptible to?
You must Login or Register to add cards