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Gen Path > Exam 2 Chapter 4 - Immune Deficiency Disorders > Flashcards

Exam 2 Chapter 4 - Immune Deficiency Disorders Flashcards

1
Q

What type of disorder causes vulnerability to infection or disease?

A

immune deficiency disorder

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2
Q

Immune deficiency disorders are either _______ or ________

A

primary (congenital) or secondary (acquired)

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3
Q

Which type of Immune deficiency disorder is rare, most commonly genetic, and has early onset (6-24 months)?

A

primary

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4
Q

X-Linked agammaglobulinemia typically occurs in _ month old _____

A

6, males

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5
Q

______ B cell maturation is a characteristic of X-Linked agammaglobulinemia

A

failed

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6
Q

Bacterial or viral infection such as otitis media, pharyngitis, and sinusitis are characteristic of which primary IDD?

A

X-Linked agammaglobulinemia

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7
Q

What is the treatment for X-Linked agammaglobulinemia?

A

IgG infusions (good prognosis)

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8
Q

Which primary IDD (Immune deficiency disorder) has an decreased antibody response to infection?

A

Common variable immunodeficiency

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9
Q

Common variable immunodeficiency has normal _ cells, but no ______ cells, and increased GI infections?

A

B, plasma

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10
Q

Which primary IDD affects males and females (10-30 yrs, 1:50,000)?

A

Common variable immunodeficiency

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11
Q

What is the most common primary IDD (Immune deficiency disorder)?

A

Isolated IgA deficiency

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12
Q

Isolated IgA deficiency has ________ IgA production and is ___________ or very mild?

A

decreased, asymptomatic

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13
Q

Isolated IgA deficiency can cause what 2 issues?

A

recurrent diarrhea or URTIs

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14
Q

How common is Isolated IgA deficiency?

A

1 in 700 caucasians

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15
Q

Hyper-IgM Syndrome is characterized by _______ IgM levels, and _______ IgG, IgA, IgE

A

increased, decreased

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16
Q

Hyper-IgM Syndrome is also characterized by _______ ______ infection?

A

recurrent bacterial

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17
Q

Which primary IDD is 70% X-linked mutations and occurs in 1:100,000 people?

A

Hyper-IgM Syndrome

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18
Q

Which primary IDD is also known as “bubble boy”? (vulernable to ALL microbial infections)

A

Severe combined immunodeficiency (SCID)

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19
Q

Severe combined immunodeficiency (SCID) is charactized by _______ and _______ atrophy

A

lymphopenia and lymphoid

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20
Q

David Vetter is associated with which primary IDD?

A

Severe combined immunodeficiency (SCID)

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21
Q

X-Linked agammaglobulinemia, Common variable immunodeficiency, Isolated IgA deficiency, Hyper-IgM Syndrome, Severe combined immunodeficiency (SCID) are the 5 ________ IDDs?

A

primary

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22
Q

Are primary or secondary IDDs more common?

A

secondary (acquired)

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23
Q

Infections, malnutrition, aging, cancer, and

Therapy-induced immunosuppression are all which type of IDD?

A

secondary

24
Q

What is the most common type of secondary IDD?

A

Therapy-induced immunosuppression

25
Q

Secondary IDDs suppress ______ and cause ________ dysfunction

A

marrow, lymphocyte

26
Q

Blood, semen, vaginal fluid, and breast milk are all ways to spread ____?

A

HIV (Human immunodeficiency virus)

27
Q

What condition destroys CD4+, T cells, and macrophages via apoptosis or cytotoxicity?

A

HIV

28
Q

Opportunistic infections, tumors, & CNS defects often affect people with what condition?

A

HIV

29
Q

AIDS is most commonly spread ____-____ sex in the US and ____-_____ sex in Africa/Asia

A

male-male, male-female

30
Q

What percentage of AIDs is sexually transmitted?

A

> 75%

31
Q

Having other STDs ________ the spread of AIDS

A

increases

32
Q

I.V drug abusers, needlestick injury, or transfusion recipients could acquire AIDS through _________ transmission

A

parenteral

33
Q

What are the 3 types of AIDS?

A

acute, chronic, crisis

34
Q

Which type of AIDS is characterized by pyrexia, pharyngitis, myalgia, or viremia?

A

acute

35
Q

Which type of AIDS is onset 2-10 years after infection?

A

chronic

36
Q

Generalized lymphadenopathy is the most common feature of what type of AIDS

A

chronic

37
Q

Chronic AIDS is characterized by a steady decrease of ___ and _ cells and ______ viremia

A

CD+ and T cells, increase

38
Q

What type of AIDS is characterized with ≤200 cells/µL, ↑ viremia, cachexia, fatigue, diarrhea, weight loss, and CNS defects?

A

crisis

39
Q

Opportunistic infections account for what percentage of AIDS related deaths?

A

80%

40
Q

Pneumocystosis and candidiasis are what type of virus/infection?

A

fungal

41
Q

KSHV, EBV and HPV are what type of virus/infection?

A

ontogenic

42
Q

What is common treatment for AIDS?

A

anti-retroviral meds

43
Q

Misfolded, mutated or unstable proteins can cause many inflammatory/inherited conditions known as what?

A

Amyloidosis

44
Q

In failed __________ Amyloid proteins accumulate, damaging tissues and disrupting function

A

phagocytosis

45
Q

Congo red dye tests for what? What is the positive indication?

A

Amyloid, apple-green color

46
Q

Electrophoresis tests what 2 substances?

A

Serum and urine

47
Q

What are the 3 types of Amyloid proteins?

A

1) amyloid light (AL)
2) amyloid associated (AA)
3) β-Amyloid (Aβ)

48
Q

B cell proliferation (plasma cells), immunoglobulin light chains, Bence-Jones proteins are characteristics of which type of amyloid protein?

A

amyloid light (AL)

49
Q

Liver: serum-associated amyloid (SAA) and chronic inflammation: TB, RA, AS, IBD, osteomyelitis are characteristics of which type of amyloid protein?

A

amyloid associated (AA)

50
Q 
Amyloid precursor protein: cerebral plaques
Alzheimer disease (APP, Down syndrome) are characteristics of which type of amyloid protein?
A

β-Amyloid (Aβ)

51
Q

What are the 3 types of Amyloidosis?

A

systemic, localized, hereditary

52
Q

Malignant plasma cells are characteristic primary or secondary amyloidosis?

A

primary

53
Q

Which type of amyloidosis is rare, familial, and geographic?

A

hereditary

54
Q

What is the most common organ affected by amyloidosis (localized)?

A

kidney

55
Q

Weakness, fatigue, cachexia are the most common features/side-effects of _______?

A

Amyloidosis

56
Q

Amyloidosis is most likely to be lethal if it involves what 2 organs?

A

kidney and heart

57
Q

Which type of amyloidosis has a poor prognosis?

A

systemic

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