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Immune Deficiency Diseases
Terms in this set (35)
What are causes of PRIMARY immune deficiency?
Inherited defects of immune system
What are causes of SECONDARY immune deficiency?
Infections, malnutrition, cancer, cytotoxic drugs, irradiation, surgery
Phagocytic cell dysfunction is commonly linked to what oral disease?
What is another name for X-linked agammaglobulinemia?
How many B cells do you have in agammaglobulinemia?
B cells are absent-low
Agammaglobulinemia is caused by mutation of which gene?
btk gene (Bruton tyrosine kinase)
Compared to a normal lymph node biopsy, X-linked agammaglobululinemia has no?
Follicles (because no B cells)
What causes common variable immunodeficiency?
Failure of terminal
differentiation of B cells to plasma cells
(low serum Igs, seen early in life, T cell defect, B cell defect, or both)
What is the clinical presentation of primary antibodies deficiencies?
- Recurrent pyogenic bacterial infection: pneumonia, otitis media.
What is the treatment for primary antibody deficiencies?
Gamma globulin (pooled human IgG) IV~monthly
Are live vaccines contraindicated or indicated against primary antibody deficiencies?
What are the clinical characteristics of primary antibody deficiency?
- mostly asymptomatic
- increased bacterial respiratory infection
- eczema-like rashes, allergies
- rx antibiotics as needed
What is the most common immunodeficiency?
Selective IgA deficiency
Rate of IgA immunodeficiency in population?
1 in 500
Mechanism of IgA immunodeficiency?
Which primary antibody deficiency is usually X-linked?
Why does Hyper-IgM Syndrome occur?
Due to a T cell defect
What is lacking in Hyper-IgM syndrome?
Lack of CD40 ligand on T cells = FAILURE to class switch
What causes DiGeorge syndrome (Thymic aplasia)? How do you get it?
deficient development of the
thymus and parathyroid
, it is a chromosomal abnormality where
What are some characteristics of DiGeorge syndrome?
- Low to
very low T cells
that results from hypocalcemia secondary to hypoparathyroidism
- facial dysmorphism
- cleft palate (50%)
Treatment for DiGeorge syndrome?
Genetics of Severe Combined Immune Deficiency?
60% are X-linked
cytokine receptor mutations,
are adenosine deaminase
What is different in Severe Combined Immune Deficiency?
T and B cells are very low
Treatment for Server Combined Immune Deficiency?
Gene therapy, bone marrow graft
What is the main cause of periodontitis?
What causes Chronic Granulomatous Disease?
(impaired oxidative burst, intracellular survival of bacteria)
What disease is caused by a deficiency in C1 inhibitor (dissociates C1r + C1s from C1q)?
What disease is caused by a deficiency in C1, C2, C4?
Immune complex disease
What disease is caused by a deficiency in C3?
Recurrent Bacterial infections
What disease is caused by a deficiency in C5-C9?
Recurrent neisserial infections
Most common primary immunodeficiency syndrome?
Selective IgA deficiency
What is the most common cause of secondary immune deficiency worldwide?
What are some effects of protein-calorie malnutrition?
T cell count
- reduced lymphocyte
- skin test
- humoral immunity mostly
what disease causes patients to become susceptible to infections of
organism such as streptococcus pneumoniaa?
Sickle cell disease
What is characteristic of multiple myeloma?
- Plasma cell malignancy
- monoclonal gammopathy
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