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Immuno-Sero LEC > Immunodeficiency Disorders > Flashcards

Immunodeficiency Disorders Flashcards

1
Q

is when the immune system errs by failing to protect the host from disease-causing agents or from malignant cells.

A

Immunodeficiency

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2
Q

Immunodeficiency

• Classification

A
  1. Based on the cause
  2. Based on component affected
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3
Q

Classification

  1. Based on Cause
A
  • Primary Immunodeficiency
  • Secondary Immunodeficiency
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4
Q

Based on Cause

• conditions resulting from a genetic or developmental defect in the immune system

A

Primary Immunodeficiency

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5
Q

Based on Cause

• loss of immune function and results from exposure to various agents

A
  • Secondary Immunodeficiency
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6
Q

Disease-carrying gene(s)

Child born with IgA deficiency, CVI, SCID, or others

A

primary immune deficiency

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7
Q

Secondary

  • Intravenous (IV) drug abuse unsafe sexual activity, and other risk behaviors
    • Mainutrition
    › Chronic diseases (such as diabetes)
    • Medications (such as corticosteroids)

Development of AIDS or other secondary immune deficiency

Impaired (weakened immune response

A

Secondary Immune Deficiency

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8
Q

Classification

Based on Component Affected

A
  • B cell
  • T cell
  • Phagocyte
  • Complement
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9
Q

• Encapsulated bacteria (S. pneumoniae)

• AIDS (affects CD4 Thelper cells)

• Pyogenic infections (i.e. caused by bacteria)

• Lack of MAC (Neisserial infections)

A
  • B cell
  • T cell
  • Phagocyte
  • Complement
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10
Q

Types of Immunodeficiencies

A

B Cell
T cell
Combined B & T
Phagocyte
Complement

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11
Q

B Cell

A

Bruton’s X-linked Aggamaglobulinemia

Common Variable Immunodeficiency
Disorders

Transient Hypogammaglobulinemia
of the Newborn

Hyper IgM

Hyper IgE

IgA deficiency

Isolated IgG Subclass Deficiency

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12
Q

T cell

A

DiGeorge Syndrome

Purine-nucleoside phosphorylase deficiency

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13
Q

Combined B & T

A

Severe Combined Immunodeficiency

a. Bare Lymphocyte Syndrome
b. X-linked SCID
c. Adenosine Deaminase Deficiency

Wiskot-Aldrich Syndrome

Ataxia Telangiectasia

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14
Q

Phagocyte

A

Chronic Granulomatous Disease

Chediak-Higashi Syndrome

Leukocyte Adhesion Defect

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15
Q

Complement

A

МАС Deficiency

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16
Q

Bruton’s X-Linked Agammaglobulinemia
- - discoverer

A

Dr. Ogden Bruton

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17
Q
  • Is a rare genetic disorder that affects the body’s ability to fight infection
  • Absence of BTK gene that codes for enz (Bruton’s tyrosine kinase)
A

Bruton’s X-Linked Agammaglobulinemia

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18
Q
  • Immature B cells will not undergo maturation due to lack of enzyme
  • Symptoms appear after 6-9 months of age
A

Bruton’s X-Linked Agammaglobulinemia

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19
Q

Recurrent fungal, protozoan, viral
infections

Recurrent bacterial infections

A

Bruton’s X-Linked
Agammaglobulinemia

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20
Q
  • enzyme needed for differentiation into a mature B cell
A

Tyrosine kinase

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21
Q
  • Failure of mature B cells to become plasma cells
  • Unknown mechanism (idiopathic)
A

Common Variable Immunodeficiency (CVI)

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22
Q

CVI

  • Congenital presentation; _____\deficiency
A

IgA and IgG

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23
Q

● Failure of the mature B cells to respond to T cells
thus they cannot differentiate into plasma cells

A

Common Variable Immunodeficiency

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24
Q

CVI DETECTION

  • (+) B cell:
A

• CD19 and CD20
• Nylon test
• Electron microscopy
• Mitogen (LPS & DEXTRAN)

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25
Q
  • LOW ANTIBODIES
  • HYPOGAMMAGLOBULENEMIA OF 2 OR MORE IMMUNOGLOBULIN ISOTYPE
A

CVI

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26
Q
  • Failure of B cells to produce antibodies due to delayed maturation of T cell in thymus
A

Transient Hypogammaglobulinemia

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27
Q
  • Also referred to as Pediatric
    Hypogammaglobulinemia
A

Transient Hypogammaglobulinemia

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28
Q
  • Starts at 4 months and upon clearance of maternal IgG, there is persistently lower IgG levels that can last up to 3 years
A

Transient Hypogammaglobulinemia

29
Q

T cell-independent B cell activation

_____secretion

A

IgM

30
Q

T cell-dependent B cell activation

____secretion

A

IgG

31
Q
  • Increased levels of IgM
  • Low levels of all other isotypes: IgG, IgA, IgD, IgE
A

X-Linked Hyper IgM Syndrome

32
Q

X-Linked Hyper IgM Syndrome

  • Absence of____ ligand on Thelper cell
  • No class switching
A

CD40

33
Q

CD40 present on the surface of B cells will react with CD40 ligand on Thelper cells to initiate class switching of____ to_____

A

IgM to IgG

34
Q
  • Impaired IL-2 production
  • Hyperactive Th2
  • Prone to allergic reactions
A

Hyper IgE

35
Q
  • Most common primary immunodeficiency
  • Failure of B cells to become IgA-producing plasma cells
A

IgA deficiency

36
Q
  • Unknown mechanism (idiopathic)
  • Recurrent gastrointestinal and respiratory infection
A

IgA deficiency

37
Q

Isolated IgG Subclass Deficiency
- Protein antigens:

A

IgG1 and IgG3

38
Q

Isolated IgG Subclass Deficiency

  • Carbohydrate antigens:
A

IgG2

39
Q

IgG Subclass Concentration in the Blood

IgG1
IgG2
IgG3
IgG4

A

70%
20%
6%
4%

40
Q

T Cell Immunodeficiency

  • 22q11.2 deletion syndrome
  • Absence of chromosome 22q
A

DiGeorge Syndrome / DiGeorge Anomaly

41
Q

T Cell Immunodeficiency
DiGeorge Syndrome / DiGeorge Anomaly
-______ deletion syndrome
- Absence of chromosome___

A

22q11.2

22q

42
Q
  • Impaired development of 3rd and 4th pharyngeal pouch which will give rise to:
    • Hard palate
    • Part of aorta
    • Parathyroid gland
    • Thymus
A

DiGeorge Syndrome / DiGeorge Anomaly

43
Q

DiGeorge Syndrome / Anomaly
CATCH 22

A

Cleft palate
Abnormal faceii (monkey)
Thymic hypoplasia
Cardiovascular disorder
Hypocalcemia

44
Q
  • When DNA is broken down=production of purines
A

Purine-nucleoside phosphorylase deficiency (PNP Deficiency)

45
Q
  • Impaired metabolism of purines due to mutations in the PNP gene results to toxic purine metabolites
A

Purine-nucleoside phosphorylase deficiency (PNP Deficiency)

46
Q
  • Accumulation of deoxyguanosine triphosphate (dGTP)
  • Number of T cells progressively decreases
A

Purine-nucleoside phosphorylase deficiency (PNP
Deficiency)

47
Q
  • S/S:
    • Neurologic disorders
    • Recurrent or chronic pulmonary infections, diarrhea, urinary tract infections (UTI), skin infections
A

Purine-nucleoside phosphorylase deficiency (PNP Deficiency)

48
Q

Severe Combined Immunodeficiency
(SCID)
- 3 SCID Types:

A

a. Bare Lymphocyte Syndrome

b. X-Linked SCID

c. Adenosine Deaminase Deficiency

49
Q
  • absence of Type I (MHC Class I) Type II (MHC Class Il)

aka TAP2 deficiency

A

Bare Lymphocyte Syndrome

50
Q

_____SCID - absence of receptors for IL-7 and IL-15

A

X-Linked

51
Q

mutations in the ADA gene that codes for adenosine deaminase enzyme

A

Adenosine Deaminase Deficiency

52
Q

→ CD43 is present in lymphocytes and platelets

A

Wiskott-Aldrich Syndrome (WAS)

53
Q

abnormality in integral membrane (CD43) ;

A

Wiskott-Aldrich Syndrome (WAS) -

54
Q

gene responsible for defect:(Wiskott-Aldrich Syndrome)

A

WASp gene

55
Q

Triad of WAS

A

• Decreased platelet survival
• Abnormal skin function (eczema)
• Defects in T and B cell function

56
Q

Aka______ is a neurodegenerative inherited disorder;

absence of ATS gene which codes for ATM protein

A

Ataxia-Telangiectasia (AT) Syndrome - aka
Louis-Bar syndrome

57
Q

gene product is a protein with multiple functions.

One of these is assisting cells to recognize damaged DNA for its repair.

In the nucleus, the product also helps control rate of cell growth and division

A

ATM

58
Q

Mutated____ leads to hypersensitivity of cells to radiation because damaged DNA
accumulates instead of being
repaired.

Unregulated cell growth leads to tumor formation.

Cells in the cerebellum die affecting coordinated movement.

In the eye the conjunctiva have small dilated blood vessels (telangiectasias).

Oculomotor apraxia occurs.

A

ATM

59
Q
  • absence of NADPH oxidase; suppurative infection
A

Chronic Granulomatous Disease (CGD)

60
Q

• Semiquantitative assay
* Tests the phagocytic function of polymorphonuclear leukocytes (PMNL)

• Cells reduce a colorless NBT into a blue-black deposit within the cell

• In CGD, the PMNLs will not turn blue

A

Nitro Blue-Tetrazolium Test (NBT)

61
Q
  • absence of LYST gene (lysosomal trafficking) which codes for LYST protein, important in the fusion of lysosome with phagosome
A

Chediak-Higashi Syndrome

62
Q

Chediak-Higashi Syndrome - absence of
____which codes for LYST protein, important in the fusion of lysosome with phagosome

A

LYST gene (lysosomal trafficking)

63
Q

Chediak-Higashi Syndrome - absence of
____which codes for LYST protein, important in the fusion of lysosome with phagosome

A

LYST gene (lysosomal trafficking)

64
Q

ALBINISM -______ helps transport melanin into melanocytes

A

lyst protein

65
Q

Leukocyte Adhesion Defect / Deficiency
(aka Lazy Leukocyte Syndrome)

A
  • Type I: absence of integrin
  • Type Il: absence of L-selectin (CD62L) and Sialyl-Lewis X-modified glycoprotein (CD15s)
66
Q

— Also absence of CD18/CD11 adhesion receptors on T cells, neutrophils, and monocytes

  • Results in abnormal adhesion, motility, and chemotaxis of cells
A

Leukocyte Adhesion Defect / Deficiency

67
Q

— S/S:
• Delayed wound healing
• Chronic skin infections

A

Leukocyte Adhesion Defect / Deficiency

68
Q

Complement Component Deficiency
• Lack of______
• No cytolysis of (intracellular) pathogen
• Recurrent infection

A

MAC (C5b6789)

Immuno-Sero LEC (30 decks)

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