robbypowell's version from 2015-12-09 00:51


Question Answer
_____ immunodeficiency is usually genetic/congenitalPrimary
_____ immunodeficiency is usually acquiredSecondary
Immunodeficiency can be brought about by affecting cell ___, ____ or ____Type, Numbers, or Function

Primary Immunodeficiency



Question Answer
Agammaglobulinemia & Hypogammaglobulinemia are caused by affected ____ cellsB cells
DiGeorge Syndrome & Wiskott Aldrich Syndrome are caused by affected ____ cellsT cells (don't forget WAS has affected platelets)
B-cells are of what lineage?Lymphoid
T-cells are of what lineage?Lymphoid
What protein plays a crucial role in B-cell development and is defective in X-linked Agammaglobinemia?Bruton's Tyrosine Kinase (BTK)
X linked disorder that Affects platelet numbers/function, Affects T cell function & affects Cytoskeleton of lymphocytesWiskott Aldrich Syndrome (T-cell system)
Neutrophil function disease that Affects Neutrophil’s ”Oxidative Burst”Chronic Granulomatous Disease (CGD)
Chronic Granulomatous disease is commonly caused by ___ ____ deficiency... which receptor?Leukocyte Adhesion Deficiency; CD18
Lack of Thymus development and neonatal Hypocalcemia (often with Heart defects esp. Teratology of Fallot & persistent truncus arteriosus)DiGeorge anomaly
Hereditary Angioedema attack is associated with condition?Complement deficiency
Recurrent bacterial infections in first year of life is indicative of what condition?X-linked Bruton's Agammoglobinemia
Hypogammaglobulinemia can progress to what if not resolved?Agammaglobulinemia (goes from LOW to NO immunglobulin)
These subclasses of IgG's are produced against proteinsIgG 1 & 3
These subclasses of IgG's are produced against carbohydrates & polysaccharidesIgG 2 & 4
Which is rarer: T cell system disease or B cell system disease?T cell is rarer
Which is harder to treat: T cell system disease or B cell system disease?T cell is harder to treat (B cells you can replace or supplement with Immunoglobulins)
Primary treatment of DiGeorge syndrome (if caught early on)Thymus transplant
***condition where CD43 expressing cells are affected, mainly platelets***Wiskott-Aldrich syndrome
T cells are very dependent on what molecule for purine metabolism?PNP (Purine Nucleoside Phosphorylase)
Term: Inability to coordinate movementsAtaxia
Reduced IgG2, IgA, IgE and T-cell #'s with DNA instability (and with inability control movements of body)Ataxia Telangiectasia (aka Louis–Bar syndrome) (neurodegenerative)
What is the most common defect of Neutrophil function?Chronic Granulomatous Disease (x-linked recessive)
What are 3 X-linked recessive immunodeficiencies we learned about in this 3 lecture?Chronic Granulomatous disease, Wiskott-Aldrich syndrome, Bruton's Agammaglobulinemia
Nitro Blue test is used to identify what disease?Chronic Granulomatous disease
Normal cells look like what with Nitro Blue test? Abnormal cells?Normal = Blue; Abnormal = not Blue (tests for oxidative burst function)
Major complement system component that causes serious problems if not functioning is ___ (it starts it all)C3

from Table 16-1 (name condition) Defects of B cell & T cell system

Question Answer
Deficiency of All antibodies especially IgG; caused by "slow development of helper function"Transient Hypogammaglobulinemia ( of infancy)
Deficiency of IgA; caused by defective IgA differentiation of B cells (some with reduced IgG2 also)IgA deficiency (not a fancy name)
Deficiency of All antibody type (X-linked disease); Pre-B cells do not differentiateX-linked Bruton's Agammaglobulinemia
Deficiency of antibodies with reduced # B cells and increased # T cells (esp CD8+)Common Variable Immunodeficiency CVID (can have many or no clear cause, apparently)
Deficiency of numbers and T cell response (caused by genetic deletion --> deficient Thymus) often associated with Heart defectsDiGeorge Syndrome
Condition can be caused by Various genetic defects; No T-Cell Receptors or defective T-Cell Reptors; Defective InterLeukine-2; failure of B cells to mature(T-cel) Severe Combined Immunodeficiency SCID
Deficiency of IgM, platelet defect, and T-cell defect (CD43 expression defective/deficient)Wiskott-Aldrich syndrome

Relevant Tests for diseases

Question Answer
CH50 & Serum C3Complement deficiency
Leukocyte Adhesion Molecule analysisPhagocyte defect (ex. Chronic Granulomatous Disease)
B cell counts and B cell proliferation in vitroB cell system diseases (e.g. CVID, Bruton's Agammaglobinemia & Hypogammaglobinemia)
T cell count and T cell functions in vitroT cell system diseases (e.g. DiGeorge, SCID)
NBT- nitro blue testChronic Granulomatous disease (tests for Oxidative Burst function)

defect with cell and associated condition (slide 20)

Question Answer
Pluripotent stem cellReticular dysgenesis
Myeloid progenitor cellAgranulocytosis
Neutrophil or Monocyte (phagocytic cell)Chronic Granulomatous disease (neutrophil) & Leukocyte Adhesion deficiency (CD18) (Neutrophil & Monocyte)
defect with maturation of B-cellsX-linked Bruton's Agammaglobinemia
defect with maturation of both B cells and T-cellsSevere Combined Immunodeficiency (SCID)
Defective or deficient Thymus neonatally or congenitallyDiGeorge syndrome
Defective function of T-cells --> failure to activate B cells to memory cellsWiskott-Aldrich syndrome

Secondary Immunodeficiency


Question Answer
4 possible secondary causes for ImmunodeficiencyDrugs, Irradiation, Cancer, AIDS
AIDS causes immunodeficiency by affecting what cells?T helper cells
Attack by our own CD8+ cells is associated with what disease?AIDS
4 opportunistic "infections" associated with AIDSKaposi's Sarcoma, Cytomegalovirus, Candia (fungal), & Pneumocystitis carinii
T/F: HIV is evolving into milder form?True (apparently) (YAAAAAY)

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