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is lec midterms > Immunodeficiency DIsorders > Flashcards

Immunodeficiency DIsorders Flashcards

1
Q

disorders in which part of the body’s immune system is missing or dysfunctional

A

immunodeficiencies

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2
Q

primary immunodeficiency

A

inherited

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3
Q

secondary immunodeficiency

A

acquired

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4
Q

results in pyogenic infections (particularly, upper and lower respiratory tract, recurrent sinusitis and otitis media are common)

A

Defects in Humoral immunity

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5
Q

results in recurrent infections with intracellular pathogens (viruses, fungi, and intracellular bacteria)

A

Defects in T-cell mediated immunity

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6
Q

results in recurrent pyogenic infections or impaired wound healing

A

Defects in Neutrophil function

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7
Q

increased risk of overwhelming bacterial infection

A

Defects in macrophage function

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8
Q

results in recurrent bacterial infections and autoimmune-type manifestations

A

Defects in Complement system

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9
Q

Age-adjusted rates of malignancy in patients with immunodef dxs are?

A

10 to 200 times greater

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10
Q

most common congenital immunodeficiency

A

Selective IgA deficiency

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11
Q

Most are asymptomatic, but those with symptoms have infections in
respiratory and gastrointestinal tract

A

Selective IgA deficiency

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12
Q

in Selective IgA deficiency if the serum is lower than ? the deficiency is considered ?

A

5mg/dL, severe

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13
Q

in selective IgA deficiency, ? specifically directed against IgA are produced by 30% to 40% of patients with severe IgA deficiency

A

IgE

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14
Q

in selective IgA deficiency it can cause anaphylactic reaction when ? containing products are transfused

A

IgA

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15
Q

BTK stands for?

A

X-linked Bruton’s Tyrosine Kinase (Btk) Deficiency or Bruton agammaglobulinemia

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16
Q

Develop recurrent bacterial infections and Lack circulating mature CD19+ B cells and deficiency or lack of all Ig class

A

X-linked Bruton’s Tyrosine Kinase (Btk) Deficiency

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17
Q

BTK is differentiated from?

A

transient hypogammaglobulinemia of infancy by the absence of CD 19+ B cells

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18
Q

BTK is exclusively for? (males or females)

A

males

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19
Q

No mature B cells, but has pre-b cells on BM

A

X-linked Bruton’s Tyrosine Kinase (Btk) Deficiency

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20
Q

most serious of the congenital immunodeficiencies

A

Severe Combined Immunodeficiencies (SCID)

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21
Q

group of related diseases that affect all T- & B-cell function with differing causes

A

Severe Combined Immunodeficiencies (SCID)

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22
Q

in BTK a group of related diseases that affect all T- & B-cell function with differing causes. what is the most common cause?

A

mutation in IL2RG gene on X-chromosome

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23
Q

Normal signaling cannot occur in cells with defective receptors, halting natural maturation

A

X-linked Bruton’s Tyrosine Kinase (Btk) Deficiency

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24
Q

who is the boy in the bubble?

A

David Phillip Vetter

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25
Q

what did David Phillip Vetter experimented on?

A

Experimental bone marrow transplant from her sister cause his death

26
Q

A X-linked recessive syndrome

A

Wiskott-Aldrich syndrome (WAS)

27
Q

WAS is defined by a triad of?

A

immunodeficiency, eczema, thrombocytopenia

28
Q

in WAS what is the primary molecular defect?

A

CD43

29
Q

lethal in childhood because of infection, hemorrhage, or malignancy

A

Wiskott-Aldrich syndrome (WAS)

30
Q

lab findings in WAS

A

decrease platelet count and size, prolonged bleeding time, absence of isohemagglutinins

31
Q

developmental abnormality of the 3rd and 4th pharyngeal pouches that affects thymus development in embryo

A

Digeorge anomaly

32
Q

The immunodeficiency associated with DiGeorge anomaly is a

A

qualitative defect in thymocyte

33
Q

Most patients show a deletion in region q11 of chromosome 22, although this anomaly is not required for diagnosis

A

Digeorge anomaly

34
Q

treatment for Digeorge anomaly

A

fetal thymus transplantation

35
Q

Caused by mutation in LYST gene

A

Chediak-Higashi syndrome

36
Q

Reduced number of NK cells and neutrophils, as well as an increased
production of inflammatory proteins; PBS shows granulocytic inclusions

A

Chediak-Higashi syndrome

37
Q

impaired fusion of lysosomes with phagosomes

A

Chediak-Higashi syndrome

38
Q

what is affected in Chediak-Higashi syndrome

A

lysosomes and melanosomes

39
Q

what category is Chediak-Higashi syndrome

A

category 4 PID

40
Q

what category is Chronic Granulomatous disease (CGD)

A

category 5 PID

41
Q

X-linked or autosomal recessive syndrome that has Has recurrent suppurative infections

A

Chronic Granulomatous disease (CGD)

42
Q

in CGD In normal individuals, activated neutrophils and mononuclear phagocytes kill organisms via the?

A

respiratory burst

43
Q

Mutations in any of the four subunits of the enzyme that catalyzes the burst, namely, ? an result in CGD

A

nicotinamide adenine dinucleotide phosphate (NADPH) oxidase

44
Q

diagnosed using Nitroblue Tetrazolium Test (NBT)

A

Chronic Granulomatous disease (CGD)

45
Q

CGD is historically diagnosed using

A

Nitroblue Tetrazolium Test (NBT)

46
Q

in CGD what will fluoresce when reduced neu are activated using PMA?

A

dihydrorhodamine (DHR)

47
Q

in CGD dihydrorhodamine will fluoresce when reduced neu are activated using?

A

phorbol myristate acetate (PMA)

48
Q

a mitogen for Neu Resulting in oxidative burst (as DHR will be reduced) measured by flow cyto

A

phorbol myristate acetate (PMA)

49
Q

phorbol myristate acetate a mitogen for Neu Resulting in oxidative burst (as DHR will be reduced) measured by?

A

flow cytometry

50
Q

the leukocytes phagocytize the microorganisms, initiating an increase in oxygen uptake. so, no dark crystals appear

A

nitroblue tetrazolium slide test (NBT)

51
Q

Autosomal recessive disorder and has a deficiency in CD18 and on T cells

A

Leukocyte Adhesion Deficiency (LAD)

52
Q

a component of adhesion receptors on neutrophils and monocytes (CD11b or CD11c)

A

CD18

53
Q

diagnosed by detecting a decreased amount of the CD11/18 antigen on patient leukocytes by flow cytometry

A

Leukocyte Adhesion Deficiency (LAD)

54
Q

recurrent bacterial infection without pus formation

A

LADI

55
Q

always seen together (deficiency in CD15s or sialyl-Lewis X), immunodeficiency is milder

A

LADII and Bombay blood group

56
Q

most common presenting symptom of some complement deficiencies

A

SLE

57
Q

Deficiencies in the early complement components ? are usually associated with a lupuslike syndrome

A

C1q, C4 and C2

58
Q

Deficiencies of the later components of complement ? are often associated with recurrent Neisseria meningitidis infections

A

C5-C9

59
Q

Deficiencies in the early complement components C1q, C4 and C2 are usually associated with a?

A

lupuslike syndrome

60
Q

Deficiencies of the later components of complement C5-C9 are often associated with?

A

recurrent Neisseria meningitidis infections

is lec midterms (7 decks)

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