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Immunology > Immunodeficiency Disorders > Flashcards

Immunodeficiency Disorders Flashcards

1
Q

Primary immunodeficiency

A

Caused by mutations and. Are present at birth

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2
Q

Secondary immunodeficiencies

A

Acquired, due to diseases, environmental factors, medical intervention

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3
Q

Histological Abnormalities in B cell deficiency

A

Absent or reduced germinal centers, reduced Ig levels

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4
Q

Infectious consequences of B cell deficiency

A

Progenic bacterial infections, extracellular

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5
Q

Infectious consequences of T cell deficiency

A

Viral and other intracellular microbial infections, virus associated malignancies

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6
Q

Innate immune system infectious consequences

A

Variable, progenic bacterial infections

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7
Q

Examples of neutrophil deficiency (4)

A
  • Neutropenia
  • Defective opsonization
  • Defective killing of bacteria
  • Defect in neutrophil migration
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8
Q

Neutrophil defect of killing bacteria examples (2)

A
  • Chronic Granulomatous disease (GCD)

- Chediak-Higashi syndrome

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9
Q

Defective neutrophil migration example

A

Leukocyte adhesion deficiency (LAD)

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10
Q

Chronic granulomatous disease

A

X linked, inability to generate superoxide anion due to NADPH oxidase and cytochrome n defect, leads to recurrent infection with skin abscesses, chronic granulomas

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11
Q

Leukocyte adhesion deficiency

A

Failure of neutrophils to emigrate out of vessels and towards antigen, failure of CD8+ to bind, recurrent bacterial infection and poor wound healing

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12
Q

Examples of humoral immunodeficiency disorders (5)

A
  • X-linked (Bruton’s) Agammaglobulinemia
  • Hyper IgM syndrome
  • Transient hypogammaglobulinemia of infancy
  • Common variable immunodeficiency (CVID)
  • Selective immunoglobulin deficiency
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13
Q

X-linked (Bruton’s) Agammaglobulinemia

A
  • Absence of all Ig classes
  • Pre-B cells in marrow can’t mature
  • Due to defect in tyrosine kinase (BTK)
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14
Q

Management of Bruton’s Agammaglobulinemia

A
  • Avoid infection
  • antibiotics
  • IVIG
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15
Q

Hyper IgM syndrome

A
  • Defect in CD40 or CD40 ligand
  • Defective class switching
  • Defective memory
  • Recurrent infection
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16
Q

Transient hypogammaglobulinemia of infancy

A

IgG developmentally delayed, but B cells are normal, usually goes away by 2-3

17
Q

Common Variable Immunodeficiency (CVID)

A

Low serum levels of all ig, increased susceptibility, B cell to plasma cell transition, treat with IVIG

18
Q

Selective Ig deficiency

A
  • Deficiency in single Ig class or subclass

- IgA most common

19
Q

Di George syndrome

A

Hypoplastic thymus, recurrent infection of intracellular bacteria, fungi, and viruses
-Also pyogenic organisms due to lack of T-cell help for B-cells

20
Q

Severe Combined immunodeficiency (SCID)

A

Both humoral and cell mediated immunity are defective, patients susceptible for all infectious agents

21
Q

Examples of SCID (3)

A
  • X-linked SCID
  • Adenosine deaminase (ADA) deficiency
  • RAG1 or RAG 2 deficiency
22
Q

X-linked SCID

A

Mutated gamma chain of IL-2 receptor, most common SCID, defective T cell, B cell and NK cell development

23
Q

Adenosine deaminase deficiency

A

-Deoxyadenosine buildup damages B and T lymphocytes because of all the cell division

24
Q

RAG1 or2 deficiency

A

Cannot undergo V(D)J recombonation, no T or B cells

25
Q

Treatment of SCID

A

Hematopoietic stem cell transplantation, Graft vs host disease is a severe risk

26
Q

Secondary immunodeficiency examples (5)

A
  • HIV infection
  • Irradiation and chemotherapy treatments
  • Involvement of bone marrow by cancers (metastasis, leukemia’s)
  • Malnutrition
  • Removal of spleen
27
Q

HIV infection

A

Depletion of CD4+ helper T cells

28
Q

Immunosenescence

A

Immune system becoming less active as we age

29
Q

Gp120

A

What HIV uses to bind to CD4

30
Q

AIDS

A

Classifies as CD4+ T cell count under 200 cells/mm3

31
Q

Treatment for HIV infection

A

-HAART (highly active antiviral therapy), cocktail of at least three antivirals

32
Q

Diagnostic lab tests for HIV

A
  • Nucleic acid test
    AG(p24) and HIV ag
    -Ab only

Immunology (17 decks)

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