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Immunity > Primary Immunodeficiency > Flashcards

Primary Immunodeficiency Flashcards

1
Q

Whentheimmunesystem isunabletoprotectagainst pathogensinthe environment

A

Primary immunodeficiency

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2
Q

Theseareconditionscharacterizedbyintrinsic defectswithintheimmunesystemandarecausedby inheritedordenovo geneticdefects

A

Primary immunodeficiency

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3
Q

Primary immunodeficiency occurs in persons born with an immune system that is either absent or

A

Hampered in its ability to function

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4
Q

Mediatehumoral specificimmunitybyproducIngantibodies

A

B cells

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5
Q

NEUTRALIZEvirusesandotherorganismsbybindingtothemand preventingtheirattachmenttohostcells

A

B cells

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6
Q

B cells opsinize microbes for phagocytosis, usually with

A

Complement

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7
Q

75%ofserumIg;mainantibodyincontrollinginfections;23day half‐life

A

IgG

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8
Q

First Ig madebyfetusandbyimmatureBcells;good agglutinatingIg(clumpsorganismsforelimination)whichleadstolysis oforganisms;5dayhalf‐life

A

IgM

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9
Q

Has Jchainandsecretory piece;majorIg insecretions;6day half‐life

A

IgA

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10
Q

Low levelsinserum;primarilyfoundonBcellsasanantigen receptor;uncertainroleinserum;3dayhalf‐life

A

IgD

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11
Q

LeastcommonserumIg duetobindingtomastcellsandbasophils;involvedinallergicreactions;2.5dayhalf‐life

A

IgE

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12
Q

The most common types of primary immune deficiency diseases come from

A

Antibody deficiencies

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13
Q

Comprises the frequency and severity of infections and the duration of infections

A

Index of Suspicion

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14
Q

Poor response to antibiotics and unusual infectious agents are also part of the

A

Index of suspicion

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15
Q

React to recurrent sino-pulmonary infections

-may also help with parasitic infections of GI tract

A

B cells

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16
Q

Fight fungal and viral infections causing mucocutaneous candidiasis, persistent respiratory infections, chronic diarrhea, and failure to thrive

A

T cells

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17
Q

Fight recurrent skin infections of visceral abscesses, poor wound healing, and peridontal disease

A

Phagocytes

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18
Q

Fight bacterial infections such as Neisseria (meningitis or ghonorrhea), and sepsis

A

Late complement components

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19
Q

Fight things like severe or recurrent herpesvirus infections

A

NK cells

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20
Q

Severe Combined Immunodeficiency (SCID),Adenosine deaminasedeficiency (ADA), Purinenucleoside phosphorylasedeficiency (PNP), Reticular dysgenesis, DiGeorgesyndrome, Ataxia-telangiectasia, and Wiskott-Aldrich syndrome are all examples of

A

T cell deficiencies

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21
Q

A group of syndromes due to various gene defects that result in virtually complete lack of T and B cell function

A

Severe Combined Immunodeficiency (SCID)

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22
Q

SCID is caused by a block in T cell development that is associated with abnormal

A

B and NK cell development

23
Q

Usually all immunoglobin levels are low in

A

SCID

24
Q

Has the most common presenting symptom of life-threatening infections in the first months of life

A

SCID

25
Q

Chromosomal defect, usually a deletion that impacts end organs derived from the 3rd and 4th pharyngeal arches

A

DiGeorge syndrome

26
Q

Has symptoms of tetrology of fallot, hypocalcemia, low set ears, and cleft palate

A

DiGeorge syndrome

27
Q

Variable T cell defect caused by low CD3+/CD4+ and B cell defects

A

Ataxia Telangiectasia

28
Q

Ataxia Telangiectasia is caused by mutations in

A

ATM gene

29
Q

Provides instructions for making a protein that helps control cell division and is involved in DNA repair

A

TM gne

30
Q

X-linked recessive defect in WASP gene

A

Wiskott-Aldrich Syndrome (WAS)

31
Q

Characterized by small platelets and thrombocytopenia with petechiae and bruising and bleeding

A

WAS

32
Q

There is a high incidence of autoimmunity and lymphoid malignancy with

-Poor prognosis

A

WAS

33
Q

Agammaglobinemia, IgA deficiency, hyper IgM, and Common Variable Immunodeficiency (CVI) are types of

A

B-cell (antibody) deficiencies

34
Q

The most common primary immunodeficiency

A

Selective IgA deficiency

35
Q

Characterized by a decreae in only IgA

  • may have recurrent infections, allergies, or autoimmune diseases
  • may progress to CVA
A

Selective IgA deficiency

36
Q

In IgA deficiency, B cells express IgA, however they are of immature phenotype with the coexpression of IgM and IgD, and they cannot fully develop into

A

IgA secreting plasma cells

37
Q

An intrinsic B-cell disorder resulting from a defect in Bruton Tyrosine Kinase (BTK), which leads to maturation arrest of pre-B cells

A

Agammaglobinemia

38
Q

Agammaglobinemia is most commonly inherited as an

A

X-linked trait

39
Q

All types of immunoglobins are either markedly reduced or absent in

A

Agammaglobinemia

40
Q

In peripheral blood, patients with agammaglobinemia have nearly absent

A

B cells

41
Q

Characterized with a normal number of B cells, but low serum levels of immunoglobins

A

Common Variable Immunodeficiency (CVI)

42
Q

Many patients with CVI do not develop recurrent infections until the

A

2nd or 3rd decades of life

43
Q

Chronic Granulomatous Disease (CGD), Chediak-Higashi syndrome, Leukocyte Adhesion Deficiency (LAD), and Hyper IgE (Job’s) syndrome are disorder in

A

Phagocytes

44
Q

Mutations of any four genes that cause deficiency functioning of the phagocyte oxidase complex

  • 70% X-linked
  • 30% Autosomal recessive
A

CGD

45
Q

Patients with CGD suffer from recurrent, life threatening

A

Bacterial infections

46
Q

The most common bacterial infection in CGD is from

A

Staph. Aureus

47
Q

Rare autosomal recessive disorder caused by mutations in the LYST gene

A

Chediak-Higashi syndrome

48
Q

Makes a protein that transports materials into lysosomes

A

LYST gene

49
Q

Characterized by large abnormal granules inneutrophils, melanocytes, hair, and Schwann cells

A

Chediak-Higashi Syndrome

50
Q

One sign of Chediak-Higashi syndrome is partial

A

Oculo-cutaneous albinism

51
Q

Patients with Chediak-Higashi syndrome have a poor prognosis without

A

BM transplant

52
Q

Pre- and post-vaccination titers are part of the initial evaluation for

A

B cell disorders

53
Q

The neutrophil oxidative burst test is diagnostic for

A

Phagocytic disorders

54
Q

What is part of the initial evaluation for the complement system?

A

Total complement level (CH50)

Immunity (33 decks)

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