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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
2. Malnutrition
3. Aging
4. Autoimmune disease
5. Burns
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
2. Ataxia-Telengiectasia
List the 5 abnormalities of Phagocytosis:
1. Chronic Granulomatous Disease (CGD)
2. Myeloperoxidase deficiency
3. G-6-P deficiency
4. Chediak-Higashi
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
2. Agammaglobulinemia
What is the treatment for SCID?
Bone marrow transplant.
What are 5 methods for evaluating immunoglobulins?
1. Electrophor/immunoelectroph.
2. RID
3. EIA
4. Rate nephelometry
5. Serology
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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