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Immunology > Immunodeficiency > Flashcards

Immunodeficiency Flashcards

1
Q

______ is a process by which point mutations in viral genes cause alterations in structure of viral surface antigens; causes year-to-year antigenic variation.

A

Antigenic drift

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2
Q

Describe antigenic shift

A
  • Virus reassorts its segmented genome and radically change surface antigen
  • no one has immunity to it
  • Usually the cause of influenza pandemics
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3
Q

______ are typically the cause of epidemics, whereas ______ are typically the cause of pandemics.

A
  • Mutation; antigenic drift
  • Recombination; antigenic shift
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4
Q

Why are RNA viruses more prone to error than DNA viruses? (Ex: influenza, HIV, coronaviruses)

A

RNA polymerase does not have the proof reading mechanisms that DNA polymerase has

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5
Q

________ is when a gene in the expression site is excised and replaced by a copy of a different homologous gene

A

Gene conversion

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6
Q

Viruses are cleared by ________ T-cells

A

Cytotoxic CD8 T-cells

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7
Q

Why are neurons good sites for latent viruses?

A

Neurons express small amounts of MHC I

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8
Q

______ viruses (3) infect epithelial cells and sensory neurons and remain latent in neurons. When reactivated, it travels down the sensory neuron to epithelium.

A
  1. Herpes simplex virus (HSV)
  2. Herpesvirus varicella-zoster (chicken pox/shingles)
  3. Epstein-Barr virus (EBV)
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9
Q

How does Mycobacterium tuberculosis evade the immune system?

A
  • Prevents fusion of phagosome with a lysosome in macrophages
  • Flourishes in vesicles
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10
Q

_______ evades the immune system by enclosing itself in an impenetrable membrane-enclosed vesicle that does not fuse with other vesicles, preventing antigen processing

A

Toxoplasma gondii

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11
Q

How does Treponema pallidum (syphilis) evade the immune system?

A

Coats itself with human proteins to evade antibody binding

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12
Q

______ expresses 10 proteins that prevent stimulation of NK-cells and CD8 T-cells; is life-threatening to immunocompromised individuals

A

Cytomegalovirus (CMV)

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13
Q

What medication is commonly used to treat CMV?

A

Acyclovir

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14
Q

_____ are small bacterial proteins that can crosslink MHCII on APCs or TCR on CD4 T-cells, causing nonspecific activation of CD4 T-cells and excessive production of IL-2, INF-γ, and TNF-α

A

Bacterial superantigens

Ex: Streptococcus aureus and Streptococcus pyogenes

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15
Q

Staphylococcal superantigen like protein 7 (SSLP7) prevents ________

A
  • Monomeric IgA from delivering bacteria to phagocytes
  • Contains binding sites for Fc region of IgA and C5 complement protein
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16
Q

What is the difference between primary and secondary immunodeficiency?

A
  • Primary: inherited, causing enhanced susceptibility to infection or autoimmunity
  • Secondary: due to environmental factors (immunosuppressive drugs)
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17
Q

Which type of gene causes immunodeficiencies in children? Dominant or recessive.

A

Dominant - only one allele is required, so it will be seen sooner

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18
Q

_____ activates macrophages and is produced by NK cells (innate), TH1 CD4 T-cells, and CD8 T-cells (adaptive)

A

IFN-γ

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19
Q

X-linked agammaglobulinemia (XLA) is caused by a defect in ______(protein) required for B-cell activation and pre-B-cell development and differentiation.

A

Bruton’s tyrosine kinase (BTK)

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20
Q

Why does XLA not occur in newborns until after 6 months of age?

A
  • The newborn will still have some of the mother’s IgG
  • Will begin to get recurrent bacterial infections
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21
Q

Phagocytosis defects lead to enhanced susceptibility to ________ infections

A

Bacterial

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22
Q

Identify syndrome:

  • Mutation: CD18 subunit of CR3, CR4, and LFA-1 adhesion molecules
  • Functional effect: Defective migration of monocytes and neutrophils to infected tissues; defective uptake of opsonized pathogens
  • Clinical effect: widespread infection with encapsulated bacteria
A

Leukocyte adhesion deficiency (LAD)

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23
Q

Identify the syndrome:

  • Mutation: NADPH oxidase
  • Functional effect: Defective respiratory burst (release of ROS); Phagocytes unable to kill pathogens
  • Clinical effect: Chronic bacterial and fungal infections; granulomas
A

Chronic granulomatous disease (CGD)

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24
Q

Identify the syndrome:

  • Mutation: lysosomal trafficking regulator protein (LYST)
  • Functional effect: Defective fusion of endosomes and lysosomes; defective phagocytosis
  • Clinical effects: Recurrent and persistent bacterial infections; granulomas: damaging effects to many organs; partial albinism; peripheral neuropathy (nystagmus)
A

Chédiak-Higashi syndrome (CHS)

25
Q

In LAD-1, what would you see in a peripheral blood smear?

A
  • Neutrophilia
  • Due to inability of phagocytes to exit circulation
26
Q

What are the treatments for CGD?

A
  1. Lifelong regimens of antibiotics and antifungals
  2. IFN-γ treatment
  3. Hematopoietic stem cell transplant
27
Q

What are the 3 P’s of CHS?

A
  1. Pyogenic infections (strep/staph)
  2. Partial albinism
  3. Peripheral neuropathy
28
Q

Patients with CGD are susceptible to catalase-positive organisms (break down H2O2 and other ROS). What are some example organisms that someone with CGD would be susceptible to?

A
  • S. aureus
  • E. coli
  • Pseudomonas
  • Aspergillus
29
Q

In the classical pathway of complement activation, C1-C4 are required for elimination of _______

A

Immune complexes

30
Q

_______ deficiency results in accumulation of immune complexes in the blood and deposition of immune complexes in tissue

A

C1-C4

31
Q

In the classical pathway of complement activation, ____ promotes the efficient elimination of bacteria by phagocytes

A

C3

32
Q

______ deficiency results in increased susceptibility to encapsulated bacteria

A

C3

33
Q

What is the function of C5-C9 in complement activation?

A

Formation of the membrane attack complex

34
Q

C5-C9 deficiency results in increased susceptibility to _______

A

Neisseria infections

35
Q

Hereditary angioedema is due to a deficiency ______, which leads to unchecked activation of complement components leading to decreased C2 and C4 protein.

A

C1 esterase inhibitor (C1INH)

36
Q

How is hereditary angioedema inherited?

A

Autosomal dominant

37
Q

Why does hereditary angioedema lead to airway obstruction and swelling of the eyelids, lips, tongue, genitals, hands, feet?

A

Bradykinin, the inflammatory mediator responsible for capillary leakage, is left unchecked due to the inability to eliminate C1.

38
Q

How is hereditary angioedema treated?

A
  1. Danazol - synthetic androgen; promotes C4 synthesis and increases C1-INH
  2. Replacement therapy with C1-INH - for sudden attacks
39
Q

What form of severe combined immunodeficiency (SCID) leads to impaired common gamma chains on cytokine receptors??

A

X-linked

40
Q

What form of SCID is caused by an absence of adenosine deaminase (ADA) or purine nucleoside phosphorylase (PNP)?

A

Autosomal recessive

41
Q

Which form of SCID is more common?

A

X-linked

42
Q

In SCID that is X-linked, you will have mainly an absence of _____ cells, whereas in autosomal recessive you will have an absence of _____ cells.

A
  • X-linked: T-cells
  • Recessive: B-cells, T-cells, NK cells
43
Q

SCID results in an extreme susceptibility to ____ infections

A

ALL infections

44
Q

______ is a genetic abnormality caused by spontaneous deletion of a portion of chromosome 22, which prevents proper formation of the thymus (among other organs and structures)

A

DiGeorge syndrome

45
Q

How is DiGeorge syndrome treated?

A
  1. Antibiotics for infections
  2. Calcium supplementation
  3. Ear tubes and/or hearing aids
  4. OT & PT
  5. Hormone replacement
  6. Surgery to repair heart defects, cleft palate, or nasal speech
46
Q

_____ is an X-linked recessive disorder in the WASp gene, which plays a role in relaying extracellular signals to the actin cytoskeleton of hematopoietic cells.

A

Wiskott-Aldrich syndrome (WAS)

47
Q

What are the symptoms of WAS?

A
  1. Recurrent bacterial infections
  2. Eczema
  3. Purpura
  4. Epistaxis
  5. Splenomegaly
48
Q

In WAS, levels of Ig_ are low and Ig_ are elevated.

A
  • IgM levels are low
  • IgE and IgA are elevated
49
Q

How is WAS treated?

A

Hematopoietic stem cell transplant (HSCT)

50
Q

Hyper-IgM syndrome is characterized by and absence of _____ in the spleen and lymph nodes

A

germinal centers

51
Q

Due to decreased levels of all other Ig in hyper-IgM syndrome, patients are more susceptible to ______ infections

A

Bacterial

52
Q

Hyper-IgM is caused by a deficiency in ______, resulting in T-lymphocytes being unable to instruct B-lymphocytes to switch their production of immunoglobulins from IgM to IgG, IgA, and IgE.

A

CD40 ligand

53
Q

How is hyper-IgM syndrome treated?

A
  • Immunoglobulin replacement therapy
54
Q

What are the target cells for HIV?

A
  • CD4 T-cells
  • Macrophages
55
Q

What class of virus is HIV?

A

Exogenous retrovirus (integrated DNA does not remain permanently in the genome)

56
Q

How does HIV integrate into the host genome?

A
  • RNA is synthesized to cDNA by reverse transcriptase
  • Integrase enzyme integrates cDNA into host genome
57
Q

Where does HIV integrate into the genome?

A
  • At long terminal repeats (LTRs)
58
Q

People with _____ deficiency are naturally resistant to HIV

A

CCR5

Immunology (13 decks)

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