Glossary of 10. Immunodeficiencies
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- What are the 2 broad categories of immunodeficiency?
- 1. Primary
2. Secondary (acquired)
- What can cause primary immunodeficiency? (2)
- 1. Genetic inheritance
2. Embryogenic abnormality
- What are the 7 categories of primary immunodeficiency?
- 1. B cell deficiency
2. T cell deficiency
3. Combined immunodeficiency
4. Partly combined immunodef.
5. Phagocytic abnormality
6. Complement deficiency
7. Lymphoproliferative disease
- Causes of 2ndary Immunodeficiency include: (6)
- 1. Infection
4. Autoimmune disease
6. Side effect from immunosuppressive, radiation, or chemo.
- List the 4 B-cell deficiencies:
- 1. Bruton's X-linked Agamma-globulinemia
2. CVID (common variable immunodeficiency)
3. Selective Ig Disorders (IgA and IgG2)
4. Hyper IgM immunodeficiency
- List the T-cell deficiencies:
- 1. DiGeorge syndrome
2. Nezelof's syndrome
- List the 3 Combined immunodeficiencies:
- 1. SCID (2 types)
2. SCID+Hematopoietic hypoplasia
3. MHC Class deficiency (bare lymph syndrome)
- List the 2 Partial combined immunodeficiencies:
- 1. Wiskott-Aldrich syndrome
- List the 5 abnormalities of Phagocytosis:
- 1. Chronic Granulomatous Disease (CGD)
2. Myeloperoxidase deficiency
3. G-6-P deficiency
5. Leukocyte adhesion defect
- List the 2 Complement regulator deficiencies:
- 1. Hereditary Angioneurotic edema
2. Paroxysmal nocturnal hemoglobinuria
- List the 2 lymphoprolif diseases:
- 1. Hodgkin's lymphoma
2. Non-hodgkin's lymphoma
- what's more common; immunodeficiency or lymphoproliferative disease?
- Describe the etiology of Bruton's X-linked agammaglobulinemia:
- Patient's bone marrow HAS pre-B stem cells but they can't mature or become plasma cells.
- What is the primary result of Bruton's agammaglob?
- All Immunoglobulin classes are low or absent.
- What symptoms are characteristic of Bruton's agammaglob?
- -Acute and recurring infection by 6 mo of age.
-Autoimmune tissue diseases
- What 3 lab findings are characteristic of Bruton's agammaglob?
- 1. Decreased/absent B cells
2. Very low Immunoglobulins
3. Normal lymph count but all are T cells, none are B.
- How is Bruton's agammaglob treated?
- Antibiotics + gammaglobulin.
- What is the most common B cell deficiency?
- Selective IgA deficiency
- Describe the etiology of Selective IgA deficiency:
- IgA B cells ONLY are the immature type and can't mature into plasma cells. An autoAb to IgA may develop in some.
- What are symptoms of IgA deficiency?
- -Mucosal infections, diarrhea, genitourinary tract infections
-Autoimmune disease (SLE, RA)
- What 2 lab findings are characteristic of Selective IgA deficiency?
- 1. Very low IgA levels
2. Normal other Ig levels
- What is the treatment for sIgA deficiency?
- What is another name for DiGeorge syndrome?
- Thymic hypoplasia
- What is the etiology of DiGeorge syndrome?
- Due to error during embryogenesis the thymus, parathyroids and heart are abnormally developed.
- What symptoms are seen in DiGeorge syndrome?
- 1. Recurrent infection
2. Fish mouth, tiny jaws, ears.
3. Involuntary musc contraction
4. Cardiac abnormalities
- What is the treatment for DiGeorge syndrome?
- Thymus transplant before 14 weeks.
- What are the 2 forms of SCID?
Where is each more common?
- 1. X-linked - U.S.
2. Autosomal recessive - Swiss
- What is the etiology of SCIDS?
- -T/B cells BOTH don't mature beyond stem cells.
-No lymph cells are in the lymph nodes, thymus, spleen, etc.
- What are the resulting symptoms of SCIDS?
- Recurring microbial infections starting early in infancy.
- What are the 2 primary lab findings in SCID?
- 1. Lymphopenia
- What is the treatment for SCID?
- Bone marrow transplant.
- What are 5 methods for evaluating immunoglobulins?
- 1. Electrophor/immunoelectroph.
4. Rate nephelometry
- What are 2 ways to evaluate cell counts?
- 1. WBC w/ diff
2. Flow cytometry
- What is a way to evaluate T cell function?
- Delayed hypersensitivity skin tests.
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