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22 Cards in this Set
- Front
- Back
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Multiple myloma
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Neoplasia of plasma cell
Cell doesn't die but secretes inappropriate products (Bence-Jones proteins-in urine) Inappropriate osteoclast action More susceptible to infection |
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Reticular Dysgenesis
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Genetic/Devel. Pri. Immunodef.
Undiff. stem cells don't develop into pluripotent stem cells Result is no blood cells Not compatible with life |
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Aplastic Anemia
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Genetic/Devel. Pri. Immunodef.
Non-lymphoid stem cells blocked No RBCs, granulocytes, etc. Not compatible with life Some drugs (chloramphenicol) can cause this |
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Chronic Granulomatous Disease (CGD)
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Pri.Phagocytic cell non-lymphocytic cell immunodef.
Defective NADPH oxidase and diminished levels of cyt. B Cannot generate H2O2 within phagosome to kill bacteria |
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Chediak-Higashi Disease
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Pri.Phagocytic cell non-lymphocytic cell immunodef.
Partial albanism Defect in neutrophil chemotaxis, phagocytosis and lysosomal killing Problems in engulfment-WBCs cannot phagocytose foreign material |
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Job's Syndrome
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Pri.Phagocytic cell immunodef.
Neutrophils don't respond to chemotaxis Skin abscesses from head to toe and disease |
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Leucocyte Adhesion Deficiency (LAD)
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Pri.Phagocytic cell immunodef.
PMNs cannot leave vascular endothelium to protect tissues Inefficient pus formation Defective CD18 Infections in mouth and GI tract |
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Severe Combined Immunodeficiency Disease (SCID)
What type Immunodeficiency? What cells are missing? Associated Syndrome? Treatment? |
Lymphocytic cell line
Prim. Immunodef. Both B & T-cells missing from peripheral circulation and lymphoid tissues. Bare Lymphocyte syndrome (no markers) Infection susceptibility Treatment: BM Transplant or gene therapy |
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Ataxia-telangiectasia
Symptoms? Deficiencies? Treatment? |
Lymphocytic cell line
Prim. Immunodef. Non-immune symptoms may occur in skin (red spider veins), motor mvmts. Deficiencies in T cells, IgA, IgG2, IgG4 Treat with bone marrow transplant and treat infections, fetal thymus graft |
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Wiskott-Aldrich syndrome
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Lymphocytic cell line
Prim. Immunodef. x-linked disorder (males only) accompanied by thrombocytopenia (low platelets) Reduced IgM Treatment: BM transplant & anti-microbial therapy with infections |
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Bruton's (X-linked infantile agammmaglobulinemia)
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B-cell deficiency
Males only Absence of Igs (all serotypes) and ABO blood type isoantibodies B-cells cannot mature Lymph nodes small and tonsils are absent T-cell function normal Pyrogenic bact. infections GI and Resp. Tract infections Lack of Bcell tyrosine Kinase |
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Transient Infantile Hypogammaglobulinemia
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B-cell Deficiency
Low IgG levels...IgM and IgA normal Normal B-cell presence in periph. circ. (unlike Bruton's) Young children-Do not do immunizations Treatment: IV gammaglobulin |
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Common Variable Immunodeficiency
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B-cell Deficiency
Onset at 15-35 years B cells become unresponsive to Th cells IgA switching is most affected Females at risk for lymphomas later in life Treatment: IV immune globulin |
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Dysgammaglobulinemias
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B-cell deficiency
IgA deficiency-most common IgG defic.-Pyrogenic bact. infections IgM defic.-bact. infect. |
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DiGeorge's Syndrome
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T-cell deficiency
Lack of development of the thyroid and parathyroid See tetany at birth (hypocalcimia) Because no thymus, T-cells don't develop B-cells there but no IgG production in response to immunization Treatment: Fetal thymus graft |
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Nezelof's Syndrome
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T-cell Deficiency
Thymic dysplasia that doesn't support normal T-cell maturation Abnormal IgG synthesis Reduced cell-mediated immune response |
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Chronic Mucocutaneous Candidiasis
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T-cell deficiency
Selective defect in T-cell responsiveness to Candidiasis IgG response normal Often endocrine dysfunctions (adrenal and parathyroid) |
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Secondary Immune Deficiencies
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Leading cause
Hodgkin's Multiple Myeloma SLE (lupus) Diabetes Alcoholic cirrhosis Malnutrition Burns Splenectomy |
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What are the T-cell immunodeficiencies?
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1. DiGeorge's Syndrome
2. Nezelof's Syndrome 3. Chronic Mucocutaneous Candidiasis |
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What are the B-cell immunodeficiencies?
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1. Bruton's (X-linked Infantile Agammaglobulinemia)
2. Transient Infantile Hypogammaglobulinemia 3. Common Variable Immunodeficiency (CVID) 4. Dysgammaglobulinemias: Selective immunoglobulin deficiency |
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What are the non-lymphocytic cell immunodeficiencies?
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1. Chronic granulomatous disease (CGD)
2. Chediak-Higashi Disease 3. Job's Syndrome 4. Leucocyte Adhesion Deficiency (LAD) |
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What are the stem cell immune deficiencies?
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1. SCID (Severe combined immunodeficiency disease)
2. Ataxia-telangiectasia 3. Wiskott-Aldrich syndrome |
