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Principles of Physiopathology > Immunodeficiencies > Flashcards

Immunodeficiencies Flashcards

Covers basics of immunodeficiency diseases and studies primary and secondary immunodeficiencies

1
Q

Immunodeficiency diseases are defective host defense systems classified as… or …

A

congenital (primary) or acquired (secondary)

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2
Q

Primary immunodeficiencies are classified in what four ways?

A
  • B cell or humoral
  • T cell or cellular
  • Defects of phagocytes
  • Abnormalities of the complement system
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3
Q

People with antibody deficiency diseases are inordinately susceptible to…

A

recurrent bacterial infections

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4
Q

Bruton X-linked Agammaglobulinemia typically appears in which sex and age?

A

Boys <1 year old (manifests early)

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5
Q

Bruton X-linked Agammaglobulinemia is characterized by a lack of… in …

A

lack of immature B cells in periphery and plasma

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6
Q

Which primary immunodeficiency is a genetic defect on long arm of X chromosome rendering B cells incapable of maturation?

A

Bruton X-linked Agammaglobulinemia

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7
Q

With Bruton X-linked Agammaglobulinemia, patients develop recurrent…

A

infections of mucosal surfaces

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8
Q

What type of diagnosis is required for Bruton X-linked Agammaglobulinemia?

A

Lab diagnosis

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9
Q

DiGeorge syndrome is caused by defective development of the third and fourth pharyngeal pouches, which give rise to… and influence…

A

thymic epithelium and parathyroid glands and influence conotruncal cardiac development

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10
Q

In the absence of functional thymus… is interrupted

A

T-cell maturation is interrupted at the pre-T cell stage

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11
Q

Which cells are effected by DiGeorge Syndrome?

A

T cells

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12
Q

Those with DiGeorge Syndrome have frequent…

A

recurrent infections

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13
Q

What is Severe Combined Immunodeficiency?

A

The worst of primary immunodeficiencies; encompasses over 20 disorders associated by deficiencies in T-cell and B-cell development and function

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14
Q

Severe Combined Immunodeficiency includes…

A

X-linked, autosomal recessive

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15
Q

Patients affected by Severe Combined Immunodeficiency present in the first few months of life with…

A

recurrent, often severe infections, diarrhea, and failure to thrive

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16
Q

What is the treatment for Severe Combined Immunodeficiency?

A

Stem cell graft treatment

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17
Q

Which primary immunodeficiency leads to spider veins?

A

Immunodeficiency with ataxia telangiectasia

18
Q

Which primary immunodeficiency has increased susceptibility to infection, specifically frequent sinopulmonary infections?

A

Immunodeficiency with ataxia telangiectasia

19
Q

How does immunodeficiency with ataxia telangiectasia affect DNA, T cells, and immunoglobulins?

A

Damages DNA by affecting repair, lowers T cell numbers as well as IgG, IgA, and IgE

20
Q

Which primary immunodeficiency is an autosomal recessive disorder demonstrating increased susceptibility to infection by pyogenic bacteria?

A

Chediak-Higashi Syndrome

21
Q

Due to defect in membrane organelles, those with Chediak-Higashi Syndrome have defective…

A

fusion of lysosomes and phagosomes

22
Q

Patients with Chediak-Higashi Syndrome will have reduced ability to… leading to…

A

reduced ability to kill ingested microbes, leading to recurrent infections with pus

23
Q

Which primary immunodeficiency occurs with frequent albinism of eyes and skin?

A

Chediak-Higashi Syndrome

24
Q

What is Wiskott-Aldrich Syndrome?

A

X-linked disorder demonstrating increased susceptibility to infection, especially S. aureus

25
Q

Which cells are affected by Wiskott-Aldrich Syndrome?

A

T and B cell numbers and functions reduced as well as immunoglobulins
Platelets abnormal and reduced

26
Q

What would be the effect of abnormal and reduced platelets that come with Wiskott-Aldrich Syndrome?

A

Prolonged bleeding, easy bruising

27
Q

LAD type I has deficiencies in…
Patients have…

A

deficiencies in integrins
Patients have localized bacterial infections which may be life-threatening

28
Q

LAD type II has deficiencies in…
Patients have…

A

deficiencies in selectins
Patients life-threatening infections and severe developmental delay

29
Q

LAD I and II are deficiencies in…

A

leukocyte adhesion

30
Q

With LAD I and II, flow cytometry shows…

A

elevated WBC count with confirmation of molecular deficiency

31
Q

What is physiologic sequelae?

A

Inability to build immune system
General malnutrition, vitamin and mineral deficiency

32
Q

Ionizing radiation, chemotherapeutics, anti-inflammatory drugs, and immunosuppressants are all examples of…

A

therapeutic treatments for secondary immunodeficiency

33
Q

Ionizing radiation damages bone marrow which will affect…

A

B-cells

34
Q

What type of drug is prednisone?

A

Anti-inflammatory

35
Q

What are three infectious examples of secondary immunodeficiency?

A
  • HIV
  • EBV
  • Schistosoma
36
Q

With HIV, what aspect of the immune system is low?

A

CD4+ which is important for T-cells

37
Q

What happens when a patient is infected with EBV?

A

WBCs are transformed to cancer cells

38
Q

What is schistosoma?

A

Parasites consume immune system which can cause cancer

39
Q

Cancers like multiple myeloma, Waldenstrom’s macroglobulinemia, and leukemias and lymphomas are all examples of what type of immunodeficiency?

A

Secondary

40
Q

What is the effect of multiple myeloma?

A

Destroyed bone marrow which produces immune cells

41
Q

Multiple myeloma’s “lesser cousin” is…

A

Waldenstrom’s macroglobulinemia

42
Q

Leukemias and lymphomas are all…

A

immune cell cancers where cancer takes over the immune system

Principles of Physiopathology (36 decks)

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