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immunodeficiency Flashcards

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1
Q

What is immunodeficiency?

A

A condition where the immune system is depressed or absent, unable to mount a normal immune response.

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2
Q

What are the consequences of immunodeficiency?

A

Associated with recurrent infections due to:
* Antibody deficiency
* Phagocyte deficiencies
* Complement protein deficiencies
* Deficiency in cell-mediated immunity

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3
Q

What are the two main subtypes of immunodeficiency?

A
  • Primary or Congenital
  • Secondary or acquired
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4
Q

What characterizes primary immunodeficiencies?

A

Inherited genetic defects in immune cell development or function.

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5
Q

What characterizes secondary immunodeficiencies?

A

Loss of previously functional immunity due to factors like infection, toxicity, radiation, splenectomy, aging, malnutrition, drugs.

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6
Q

How many types of primary immunodeficiencies have been identified?

A

Over 150 different types.

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7
Q

What are the components of the immune system?

A

Stem cells split into:
* Myeloid progenitor
* Lymphoid progenitor

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8
Q

Which cells are considered the first line of defense?

A
  • Dendritic cells
  • Macrophages
  • Monocytes
  • Neutrophils
  • NK-cells
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9
Q

What is the most common type of primary immunodeficiency?

A

Antibody deficiency (50% - B cell deficiency).

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10
Q

What is Severe Combined Immune Deficiency (SCID)?

A

A group of disorders involving absent or reduced T cell function combined with disrupted antibody responses.

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11
Q

What is the frequency of gamma chain deficiency in SCID cases?

A

45.9%

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12
Q

What mutation causes X-linked SCID?

A

Mutation in the interleukin 2 receptor gamma gene (IL-2Rg).

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13
Q

What is DiGeorge syndrome?

A

A condition characterized by thymic hypoplasia or aplasia affecting T cell development.

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14
Q

What is the treatment for X-linked agammaglobulinemia?

A

Antibiotics and passively administered antibodies.

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15
Q

What causes Hyper IgM syndrome?

A

Defective CD40 ligand leading to no isotype switching.

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16
Q

What is Chronic Granulomatous Disease?

A

A phagocyte deficiency due to NADPH oxidase defect.

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17
Q

What is a common result of complement deficiencies?

A

Recurrent bacterial infections, especially meningitis.

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18
Q

What are common causes of acquired immunodeficiencies?

A
  • Cancer
  • Cytotoxic drugs or radiation
  • Malnutrition
  • Splenectomy
  • Immunosuppressive therapies
  • Stress
  • Aging
  • Infection (e.g., HIV)
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19
Q

What virus causes Acquired Immune Deficiency Syndrome (AIDS)?

A

Human immunodeficiency virus (HIV).

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20
Q

What are the phases of HIV disease progression?

A
  • Primary HIV Infection
  • Chronic Asymptomatic Phase
  • Overt AIDS
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21
Q

What is the role of CD4+ T cells in HIV infection?

A

HIV infects and destroys CD4+ T cells, leading to immune system failure.

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22
Q

What is the main treatment strategy for some forms of SCID?

A

Gene therapy to reintroduce the IL2Rg gene.

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23
Q

Fill in the blank: Immunodeficiences can arise from _______ or _______.

Study These Flashcards
A

Primary or Congenital; Secondary or acquired.

24
Q

True or False: All primary immunodeficiencies require major intervention.

25
What are the symptoms of Congenital hypogammaglobulinemia?
Symptoms typically appear at 9 months to 2 years of age.
26
What is the genetic defect in X-linked hyper-IgM syndrome?
Defective CD40 ligand.
27
What is immunodeficiency?
A state where the immune system is depressed or absent, preventing a normal immune response.
28
What happens when the immune system attacks its own cells?
Autoimmunity.
29
What infections are associated with antibody, phagocyte, or complement protein deficiencies?
Recurrent infections.
30
What are the consequences of cell-mediated immunity deficiency?
Chronic viral, fungal, or protozoal infections.
31
What are the two main types of immunodeficiency?
Primary (congenital) and Secondary (acquired).
32
What causes primary immunodeficiency?
Inherited genetic defects in immune cell development or function.
33
What causes secondary immunodeficiency?
Infection (e.g., HIV), toxicity, radiation, splenectomy, aging, malnutrition, drugs.
34
What do myeloid progenitors produce?
Dendritic cells, macrophages, monocytes, neutrophils (first-line defense).
35
What do lymphoid progenitors produce?
NK-cells (first-line), T-cells, and B-cells (adaptive immunity).
36
What percent of PIDs are antibody (B cell) deficiencies?
50%.
37
What are combined deficiencies?
B and T cell deficiencies (20%).
38
Which PIDs affect T cells only?
Cellular (10%).
39
What is SCID?
Severe Combined Immunodeficiency, characterized by absence of T and/or B cells.
40
What are the causes of SCID?
ADA/PNP deficiency, yc chain mutation, JAK3 mutation, RAG1/2 deficiency.
41
What is DiGeorge Syndrome?
Thymic aplasia due to 22q11 deletion, affecting T cell development.
42
What does X-linked hyper-IgM syndrome cause?
No isotype switching; leads to susceptibility to extracellular bacteria, *Pneumocystis carinii*, and *Cryptosporidium parvum*.
43
What is selective IgA deficiency linked to?
Respiratory infections.
44
What does complement deficiency often cause?
Susceptibility to Neisseria species infections.
45
What mutation causes X-linked SCID?
IL-2 receptor gamma (IL-2Rg) mutation.
46
What is the role of JAK3?
Cytokine signaling; its deficiency also causes SCID.
47
What are the most common SCID genetic defects?
* Gamma chain (45.9%) * ADA (16.5%) * IL-7Rα (10%).
48
What treatment showed promise but caused leukemia in trials?
Gene therapy for IL2Rg using viral vectors.
49
Which gene therapy was successful later?
ADA-SCID.
50
What is X-linked agammaglobulinemia?
Btk mutation halting B cell development.
51
What causes Chronic Granulomatous Disease?
NADPH oxidase defect, no oxidative burst.
52
What is Chediak-Higashi syndrome?
Defective lysosome formation, impaired bacterial lysis.
53
What causes AIDS?
HIV infection.
54
Which cells does HIV target?
CD4+, CCR5+, CXCR4+ T cells, monocytes, and dendritic cells.
55
What receptors are involved in HIV entry?
CCR5 (M tropism), CXCR4 (T tropism).
56
What are the stages of HIV infection?
* Primary infection * Chronic asymptomatic phase * Overt AIDS.
57
What happens during overt AIDS?
CD4 count drops, viral load increases, and opportunistic infections occur.

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