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Nelson - Immunology > Evaluation Of Suspected Immunodeficiency > Flashcards

Evaluation Of Suspected Immunodeficiency Flashcards

1
Q

Most cost-effective screening tests for immunodeficiency

A

CBC, ESR

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2
Q

Primary immunodeficiency: HypoCa, unusual facies and ears, heart disease

A

DiGeorge anomaly

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3
Q

Primary immunodeficiency: Delayed umbilical cord detachment, leukocytosis, recurrent infections

A

Leukocyte adhesion deficiency

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4
Q

Primary immunodeficiency: Persistent thrush, failure to thrive, pneumonia, diarrhea

A

Severe combined immunodeficiency

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5
Q

Primary immunodeficiency: Bloody stools, draining ears, atopic eczema

A

Wiskott-Aldrich syndrome

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6
Q

Primary immunodeficiency: P. jiroveci pneumonia, neutropenia, recurrent infections

A

X-linked hyper-IgM syndrome

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7
Q

Primary immunodeficiency: Severe progressive infectious mononucleosis

A

X-linked lymphoproliferative syndrome

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8
Q

Primary immunodeficiency: Recurrent staphylococcal abscesses, staph pneumonia with pneumatocele formation, coarse facial facies, pruritic dermatitis

A

Hyper IgE syndrome

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9
Q

Primary immunodeficiency: Persistent thrush, nail dystrophy, endocrinopathies

A

Chronic mucocutaneous candidiasis

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10
Q

Primary immunodeficiency: Short stature, fine hair, severe varicella

A

Cartilage hair hypoplasia with short-limbed dearfism

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11
Q

Primary immunodeficiency: Oculocutaneous albinism, recurrent infection

A

Chediak-Higashi syndrome

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12
Q

Primary immunodeficiency: Abscesses, suppurative lymphadenopathy, antral outlet obstruction, pneumonia, osteomyelitis

A

Chronic granulomatous disease

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13
Q

Primary immunodeficiency: Progressive dermatomyositis eith chronic enterovirus encephalitis

A

X-linked agammaglobulinemia

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14
Q

Primary immunodeficiency: Sinopulmonary infections, neurologic deterioration, telangiectasia

A

Ataxia-telangiectasia

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15
Q

Primary immunodeficiency: Recurrent neisserial meningitis

A

C6, C7 or C8 deficiency

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16
Q

Primary immunodeficiency: Sinopulmonary infections, splenomegaly, autoimmunity, malabsorption

A

Common variable immunodeficiency

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17
Q

A normal absolute lymphocyte count (ALC) result rules out against

A

T cell defect

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18
Q

A normal ANC rules out against

A

1) Congenital or acquired neutropenia 2) Both forms of leukocyte adhesion deficiency

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19
Q

Normal platelet count excludes

A

Wiskott-Aldrich syndrome

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20
Q

Evaluation of suspected immunodeficiency should be initiated in the presence of

A

1) 1 or more systemic bacterial infections 2) 2 or more serious respiratory or documented bacterial infections 3) Serious infections occurring at unusual sites 4) Unusual pathogens 5) Common pathhogens with unusual severity

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21
Q

Absence of Howell-Jolly bodies rules out

A

Asplenia

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22
Q

ESR result in chronic bacterial infection

A

Normal

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23
Q

Normal ESR result indicates that this infection is unlikely

A

Fungal

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24
Q

Screening tests for B cell defects

A

IgA measurement, if abnormal, IgM and IgG measurment

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25
Q

Candida albicans intradermal skin test is a screening test for

A

T cell defects

26
Q

Respiratory burst assay is a screeningg test for

A

Phagocytic cell defect

27
Q

CH50 is a screening test for

A

Complement deficiency

28
Q

Most common B cell defect

A

IgA deficiency

29
Q

Measurement of isohemagglutinins (anti-A and anti-B) measures predominantly what type of Ig

A

IgM

30
Q

Most cost-effective test of T cell function

A

Candida skin test

31
Q

Positive Candida skin test

A

Erythema and induration >/ 10mm at 48hrs

32
Q

Killing defect of phagocytic cells should be suspected of a patient has

A

1) Recurrent staph abscesses 2) Recurrent G- infections

33
Q

Most reliable and useful test for phagocytic cells

A

Flow cytometry after respiratory burst using rhodamine dye, which has replaced NBT dye test

34
Q

Early diagnosis of LAD is of crucial importance because

A

Stem cell transplantation is life-saving

35
Q

Most effective screening test for complement deficiency

A

CH50 assay

36
Q

CH50 assay measures

A

Intactness of the entire complement pathway

37
Q

Most common cause of an abnormal CH50 result

A

Delay in or improper transport of specimen to lab

38
Q

Measurement of this complement factor can be helpful in assessing suspected hereditary angioedema

A

C4

39
Q

B vs T vs C’ vs neutrophil defect: Recurrent pyogenic infections with extracellular encapsulated organisms

A

B cell and complement

40
Q

B vs T vs C’ vs neutrophil defect: Otitis, sinusitis, recurrent pneumonia, bronchiectasis, and conjunctivitis

A

B cell

41
Q

B vs T vs C’ vs neutrophil defect: Recurrent N meningitidis infection

A

C’

42
Q

B vs T vs C’ vs neutrophil defect: Recurrent infections with less virulent or opportunistic organisms

A

T cell

43
Q

B vs T vs C’ vs neutrophil defect: Recurrent dermatologic infections

A

Neutrophil

44
Q

B vs T vs C’ vs neutrophil defect: Increased incidence of autoimmune disease

A

C’

45
Q

B vs T vs C’ vs neutrophil defect: Diarrhea common

A

B cell

46
Q

B vs T vs C’ vs neutrophil defect: Subcu, lymph node, lung, and liver abscesses

A

Neutrophil

47
Q

B vs T vs C’ vs neutrophil defect: Pulmonary infections including abscess and pneumatocele

A

Neutrophil

48
Q

B vs T vs C’ vs neutrophil defect: Anergy

A

T cell

49
Q

B vs T vs C’ vs neutrophil defect: Minimal growth retardation

A

B cell

50
Q

B vs T vs C’ vs neutrophil defect: Susceptible to graft vs host disease from non irradiated blood or from maternal engraftment

A

T cell

51
Q

B vs T vs C’ vs neutrophil defect: Bone and joint infection common

A

Neutrophil

52
Q

B vs T vs C’ vs neutrophil defect: Delayed separation of umbilical cord

A

Neutrophil

53
Q

B vs T vs C’ vs neutrophil defect: Fatal reactions from live virus or BCG vaccination

A

T cell

54
Q

B vs T vs C’ vs neutrophil defect: Absence of pud at site/s of infection

A

Neutrophil

55
Q

B vs T vs C’ vs neutrophil defect: High incidence of malignancy

A

T cell

56
Q

B vs T vs C’ vs neutrophil defect: Poor wound healing

A

Neutrophil

57
Q

B vs T vs C’ vs neutrophil defect: Poor survival beyond infancy or early childhood

A

T cell

58
Q

Evaluation of immune function should be initiated in those rare infants or children who

A

1) Have positive family history of early infant death or known ID 2) Unusual chronic or recurrent infections such as 1 or more systemic bacterial infections (sepsis/meningitis), 2 or more serious respiratory or documented bacterial infections within 1 year, serious infections occurring at unusual sites, infections with unusual pathogens, infections with common childhood pathogens but unusual severity

59
Q

Most common B cell defect

A

Selective IgA deficiency

60
Q

T/F Antibody titers are not interpretable after the patient has received a blood transfusion

A

T

Nelson - Immunology (2 decks)

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