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immuno deficiencies
WVSOM -- Immuno/Micro -- Immunodeficiency Diseases
Question | Answer |
---|---|
What are the primary immuno deficiencies? | b-cell defects; t cell defects; combined t and b cell defects; phagocyte defects; complement defects |
What are teh antibody deficiencies? (B-Cell) | IgA deficiency; X-linked agammaglobulinaemia; IgG subclass deficiency; immunodeficiency with increased IgM; common variable immunodeficiency |
How does Tcell help for B-cell differentiation? | B cell CD40 binds to T cell CD 40 ligand (CD154) |
Explain antibody immunodeficiency with increased IgM | low igG, IgA adn increaed IgM; defect in T-cell help so there isn't differentiation. |
What is common variable immunodeficiency | CVID; occurs in 2/3 decades; no plasma cells; recurrent sinopulmonary infections |
Whoat is CVID treated? | IVIg antibodies |
What is primary immunodeficiency | due to intrinsic (congenital or acquired) defects in cells or factors of the immune system |
What is secondary immunodeficiency? | results from extrinsic factors, such as drugs, irradiation, malnutrition or infection |
What is most common cause of secondary immunodeficiency? | malnutrition |
What is seen more, primary or secondary immunodeficiency? | secondary |
What is hereditary angioedema? | C-1 inhibitor is deficient. recurrent edema of subcutaneous and submucoseal tissue. |
What does C1 inhibitor do? | activation of the clotting, kinin, plasmin, and complement systems. |
How do you diagnose an immunodeficiency? | recurrent infections; persistent or severe infections; unusual infections; paucity of lymphoid tissue; failure to thrive in infatns; skin lesions; oral and perineal candidiasis; diarrhea and malabsorption |
What is the laboratory evaluation of immune competence? | serum Ig quantitation; cbc and differential; lymphocyte counts; phagocytic index; complement activity; response to vaccination |
What is the major immunologic deficit in X linked agammaglobulinemia? | no B cells. affets male children |
what is the treatment for x-linked agammaglobulinemia? | IVIg |
What is the most commmon immunoglobulin deficiency? | IgA |
What symptoms are seen with IgA deficiency? | asymptomatic or recurrent bacterial infections |
What is severe combined immunodeficiency? | SCID; combined t and B cell defect. recurrent serious infections. fatal within 2 years w/o treatment. |
What is wiskott-aldrich syndrome | Immunodeficiency with eczeme, thrombocytopenia and recurrent infections; defects in Ig and T cells |
What are the 2 T adn B cell defects | SCID adn Wiskott-Aldrich syndrome |
What is deficient in SCID? | ADA and PNP |
Infants with SCID are prone to what types of infections? | oral candidiasis and opprotunistic diseases |
Waht treatments are available for victims of SCID | bone marrow transplant; bubbles; gene therapy |
How woudl deficiencies in MHC genes affect teh production of fucntional T cells | MCH will decide if a T cell will live or apoptose in teh thymus; deficiencies will not allow bad T cells to apoptose |
What is DiGeorge's syndrome? | thymus and parathyroid fail to devleop. |
What is the triad of DiGeorge? | hypercalcemia; dec T cells; congential heart disease |
What is the treatment for DiGeorge? | thymic tissue graft or fetal thymus transplantation |
What are extrinsic disorders of phagocytes? | defects in some other aspect of the immune or inflammatory response that diminish phagocytosis |
What are intrinsic disorders of phagocytes? | involve defects in teh intracellular killing machinery |
What is an example of an intrinsic phagocyte disorder? | chronic granulomatous disease |
What is chronic granulomatous disease? | defects in intracellular killing; granuloma formation; age of onsite is 2 years; x-linked; |
What is deficient adn defective in granulomatous disesase? | IFN-gamma and IL-12 defects and Rac2 deficiency; LEUKOCYTE ADHESION DEFICIENCY TYPES I and II; myeloperoxicase deficiency |
What is the primary defect in leukocyte adhesion deficiency? | B2 integrin defect |
What is seen in LAD? | High Blood PMN count; servere periodontitis, recurrent infections, early tooth decay |