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Immunosero Lec (Midterm) > 5.1 Immunodeficiency disorders > Flashcards

5.1 Immunodeficiency disorders Flashcards

1
Q

Defined as:

Decreased host resistance to some types of infection

Decreased in cell number of functions

Excess production of molecules whose role is to “ dampen” the immune response

A

Immunodeficiency

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2
Q

2 types of immunodeficiency

A

Primary
Secondary

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3
Q

Inherited type of immunodeficiency that is results for developmental deects

A

Primary immunodeficiency

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4
Q

2 Major mechanisms

A
  1. Defect in effector cells (Immune cells)
  2. Defect in effector molecule production in HI (Humoral) or CMI (Cell-mediated)
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5
Q

One deficiency can impair the entire system

T or F

A

T

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6
Q

Classified accrding to cell or effector that is directly affected:

B cell (50%)
T cells (10%)
Combined B and T cells (15-20%)
Phagocytes (20%)
Complement (2-4%)

T or F

A

T

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7
Q

Results from developmental defects

A

Primary immunodecificency

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8
Q

In primary immunodeficiency, the defect is usually in what cell?

A

Effector cell

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9
Q

What is the common among B cell deficiency?

When B cells disorder occurs, there is deficiency in?

A

Gammaglobulins and antibodies

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10
Q

X-linked

Defect in gene coding for Bruton’s Tyrosine kinase

Pre-B cells do not develop to mature B cels: normal CMI

Deficient in GAMMAGLOBULINS

A

Bruton’s Agammaglubulinemia

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11
Q

Bruton’s Agammaglubulinemia is more effected in what sex?

A

Male

Due to single X in there chromosomes

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12
Q

What is the enzyme defected in Bruton’s agammaglobulinemia?

A

Tyrosine kinase

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13
Q

Enzyme that is vital for the development of B cells?

A

Tyrosine kinase

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14
Q

Total plasma/serum immunoglobulin is the lab diag for?

A

Bruton’s Agammaglubulinemia

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15
Q

Most common immunodeficiency

A

Selective IgA Deficiency

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16
Q

Diminished IgA levels; B cells do not differentiate into IgA-secreting plasma cells

A

Selective IgA deficiency

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17
Q

IV therapeutic gamma globulin is used for transfusion for?

A

Bruton’s Agammaglubulinemia

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18
Q

IV therapeuutic gamma globulin is not recommended because:

1.IgA is present at high levels in plasma
2. Circulating IgA would not reach the mucosal regions
3. Secretory IgA is required
4. Patients may experience transfusion reaction (Px anti-IgA reacts with donor IgA)

Which of the following is wrong?

A

1

Low levels

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19
Q

What reaction the causes patients may anti IgA reacts with donor IgA?

A

Transfusion-associated anaphylaxis

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20
Q

IV therapeutic gamma globulin is can be used for selective IgA deficiency?

T or F

A

F

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21
Q

No memory cell formation (No isotype switching) due to defect in T cell help

X-linked

A

Hyper IgM

Immunoglobulin Deficiency with increased IgM

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22
Q

Specific immunoglobulin measurement is used for lab diag for?

A

Hyper IgM

Immunoglobulin Deficiency with increased IgM

23
Q

Low antibody levels during 5-6 moths of age due to inability to produce antibodies

Normal CMI

problem in Humoral factors

A

Transient (meaning temporary) hypogammaglobluinemia of infancy

24
Q

IgG is most affected: Ig levels normalize by 9-15 moths of age in Transient hypogammaglobluiemia

A

T

25
Q

Most common primary immunodeficiency with severe clinical manifestations

B cells do not differentiate to Ab secreting plasma

A

Common variable immunodeficiecy

26
Q

Decreased level of one or more IgG subclass

Deficiency of one subclass usually do not cause a decrease in total IgG concentration

A

Isolated IgG subclass deficiency

27
Q

In Isolated IgG subclass deficiency:

IgG1 and IgG 3 = against protein antigens

IgG2 and IgG4 = against carbohydrate antigens (Most common)

T or F

A

T

28
Q

A T cell disorder:

Congenital thymic hypoplasia

Defective embryogenesis leading to reduce and defective thymus leading to few or no circulating T cells

A

DiGeorge syndrome

29
Q

Increased susceptibility to viral, fungal, and intracellular bacterial infection due to no circulation of T cells

A

DiGeorge syndrome

30
Q

Physical malformation arising from development from development defects

A

DiGeorge syndrome

31
Q

Rare autosomal recessive trait disorder

Defect in an enzyme required in purin e metabolism

Accumulation of cytotoxic purine metabolism

A

Purine Nucleoside Phosphorylase Deficiency

32
Q

Combine B and T cell Disorders:

A
33
Q

Most serious congenital immundeficiency with severed consequences

A

Severe combined immunodeficiency

34
Q

In severe immunodeficiency, what enzyme is defect that affects purine nucleoside metabolism

A

Adenosine deaminase

35
Q

What cell is most affected in Severe combined immunodeficiency

A

T cell

36
Q

B and T cell count by flow cytometry is used to diagnose?

A

Severe combined immunodeficiency

37
Q

X-linked: defect in WAS protein (involved in cytoskeleton reorganization during B and T cell activation

A

Wiskott-Aldrich syndrome

38
Q

T cells do not respond via TCR and IL-2, what is the disease?

A

Wiskott-Aldrich Syndrome

39
Q

Autocrine is produced by? for proliferation

A

IL-2

40
Q

Total plasma/serum IgM levels and Quantitative platelet count is lab diag for?

A

Wiskott-Aldrich Syndrome

41
Q

Cerebellar ataxia and telangiectasia: rare autosomal recessive disorder

A

Ataxia-Telangiectasia

42
Q

AT gene at chromosome 11 region q22 resulting in a defective kinase involved in DNA repair and cell cycle control

A

Ataxia Telangiectasia

43
Q

Inherited defect or deficiency of NADPH oxidase complex leading to reduced ROS formation

A

Chronic Granulomatous disease

44
Q

Nitroblue tetrazolium test is used to diagnose?

A

Chronic granulomatous disease

45
Q

Determines neutrophil-mediated conversions of NRT (Colorless) to formazan (Purple precipitates

(Dark purple stains)

A

Nitroblue tetrazolium

46
Q

Mutation in the gene coding for LYST protein

Neutrophils contains Giant cytoplasmic granules

A

Chediak-Higashi Syndrome

47
Q

Defect in CD18 (Adhesion protein)

Defective diapedesis; poor pus formation and wound healing

A

Leukocyte adhesion Deficiency

48
Q

Flow cytometry using anti CD-18 monoclonal antibodies is a lab diag fr?

A

Leukocyte adhesion deficiency

49
Q

Secondary immunodeficiency:

Some viruses developed evasive to evade host immunity

A

Viral infections

50
Q

Secondary immunodeficiency:

Use of toxic drugs that intend to destroy tumor cells
Also attacks other proliferating cells
USe of immunosuppressive drugs
Result to nonspecific imunosuppression

A

Therapeutic drugs

Anti-neoplastic (Immune suppress and suppress cancer)

51
Q

Deficiencies in specific vitamins and cofactor required for optimal functioning of immune system is also supressive

A

Malnution and diseases

52
Q

Hormoal changes during pregnancy

Aging is associated with suppresion of cell mediated immunity

T or F?

A

T

53
Q

Produced in malignancies; uncontrolled production of B and T cells

Monoclonal gammopathy

T or F

A

T

Immunosero Lec (Midterm) (10 decks)

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