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22 Cards in this Set

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Multiple myloma
Neoplasia of plasma cell
Cell doesn't die but secretes inappropriate products (Bence-Jones proteins-in urine)
Inappropriate osteoclast action
More susceptible to infection
Reticular Dysgenesis
Genetic/Devel. Pri. Immunodef.
Undiff. stem cells don't develop into pluripotent stem cells
Result is no blood cells
Not compatible with life
Aplastic Anemia
Genetic/Devel. Pri. Immunodef.
Non-lymphoid stem cells blocked
No RBCs, granulocytes, etc.
Not compatible with life
Some drugs (chloramphenicol) can cause this
Chronic Granulomatous Disease (CGD)
Pri.Phagocytic cell non-lymphocytic cell immunodef.
Defective NADPH oxidase and diminished levels of cyt. B
Cannot generate H2O2 within phagosome to kill bacteria
Chediak-Higashi Disease
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
Job's Syndrome
Pri.Phagocytic cell immunodef.
Neutrophils don't respond to chemotaxis
Skin abscesses from head to toe and disease
Leucocyte Adhesion Deficiency (LAD)
Pri.Phagocytic cell immunodef.
PMNs cannot leave vascular endothelium to protect tissues
Inefficient pus formation
Defective CD18
Infections in mouth and GI tract
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
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
Wiskott-Aldrich syndrome
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
Bruton's (X-linked infantile agammmaglobulinemia)
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
Transient Infantile Hypogammaglobulinemia
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
Common Variable Immunodeficiency
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
Dysgammaglobulinemias
B-cell deficiency

IgA deficiency-most common
IgG defic.-Pyrogenic bact. infections
IgM defic.-bact. infect.
DiGeorge's Syndrome
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
Nezelof's Syndrome
T-cell Deficiency

Thymic dysplasia that doesn't support normal T-cell maturation
Abnormal IgG synthesis
Reduced cell-mediated immune response
Chronic Mucocutaneous Candidiasis
T-cell deficiency

Selective defect in T-cell responsiveness to Candidiasis
IgG response normal
Often endocrine dysfunctions (adrenal and parathyroid)
Secondary Immune Deficiencies
Leading cause
Hodgkin's
Multiple Myeloma
SLE (lupus)
Diabetes
Alcoholic cirrhosis
Malnutrition
Burns
Splenectomy
What are the T-cell immunodeficiencies?
1. DiGeorge's Syndrome
2. Nezelof's Syndrome
3. Chronic Mucocutaneous Candidiasis
What are the B-cell immunodeficiencies?
1. Bruton's (X-linked Infantile Agammaglobulinemia)
2. Transient Infantile Hypogammaglobulinemia
3. Common Variable Immunodeficiency (CVID)
4. Dysgammaglobulinemias: Selective immunoglobulin deficiency
What are the non-lymphocytic cell immunodeficiencies?
1. Chronic granulomatous disease (CGD)
2. Chediak-Higashi Disease
3. Job's Syndrome
4. Leucocyte Adhesion Deficiency (LAD)
What are the stem cell immune deficiencies?
1. SCID (Severe combined immunodeficiency disease)
2. Ataxia-telangiectasia
3. Wiskott-Aldrich syndrome