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