Evaluation Of Suspected Immunodeficiency

Question 1

Most cost-effective screening tests for immunodeficiency

Answer 1

CBC, ESR

Question 2

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

Answer 2

DiGeorge anomaly

Question 3

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

Answer 3

Leukocyte adhesion deficiency

Question 4

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

Answer 4

Severe combined immunodeficiency

Question 5

Primary immunodeficiency: Bloody stools, draining ears, atopic eczema

Answer 5

Wiskott-Aldrich syndrome

Question 6

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

Answer 6

X-linked hyper-IgM syndrome

Question 7

Primary immunodeficiency: Severe progressive infectious mononucleosis

Answer 7

X-linked lymphoproliferative syndrome

Question 8

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

Answer 8

Hyper IgE syndrome

Question 9

Primary immunodeficiency: Persistent thrush, nail dystrophy, endocrinopathies

Answer 9

Chronic mucocutaneous candidiasis

Question 10

Primary immunodeficiency: Short stature, fine hair, severe varicella

Answer 10

Cartilage hair hypoplasia with short-limbed dearfism

Question 11

Primary immunodeficiency: Oculocutaneous albinism, recurrent infection

Answer 11

Chediak-Higashi syndrome

Question 12

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

Answer 12

Chronic granulomatous disease

Question 13

Primary immunodeficiency: Progressive dermatomyositis eith chronic enterovirus encephalitis

Answer 13

X-linked agammaglobulinemia

Question 14

Primary immunodeficiency: Sinopulmonary infections, neurologic deterioration, telangiectasia

Answer 14

Ataxia-telangiectasia

Question 15

Primary immunodeficiency: Recurrent neisserial meningitis

Answer 15

C6, C7 or C8 deficiency

Question 16

Primary immunodeficiency: Sinopulmonary infections, splenomegaly, autoimmunity, malabsorption

Answer 16

Common variable immunodeficiency

Question 17

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

Answer 17

T cell defect

Question 18

A normal ANC rules out against

Answer 18

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

Question 19

Normal platelet count excludes

Answer 19

Wiskott-Aldrich syndrome

Question 20

Evaluation of suspected immunodeficiency should be initiated in the presence of

Answer 20

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

Question 21

Absence of Howell-Jolly bodies rules out

Answer 21

Asplenia

Question 22

ESR result in chronic bacterial infection

Answer 22

Normal

Question 23

Normal ESR result indicates that this infection is unlikely

Answer 23

Fungal

Question 24

Screening tests for B cell defects

Answer 24

IgA measurement, if abnormal, IgM and IgG measurment

Question 25

Candida albicans intradermal skin test is a screening test for

Answer 25

T cell defects

Question 26

Respiratory burst assay is a screeningg test for

Answer 26

Phagocytic cell defect

Question 27

CH50 is a screening test for

Answer 27

Complement deficiency

Question 28

Most common B cell defect

Answer 28

IgA deficiency

Question 29

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

Answer 29

IgM

Question 30

Most cost-effective test of T cell function

Answer 30

Candida skin test

Question 31

Positive Candida skin test

Answer 31

Erythema and induration >/ 10mm at 48hrs

Question 32

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

Answer 32

1) Recurrent staph abscesses 2) Recurrent G- infections

Question 33

Most reliable and useful test for phagocytic cells

Answer 33

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

Question 34

Early diagnosis of LAD is of crucial importance because

Answer 34

Stem cell transplantation is life-saving

Question 35

Most effective screening test for complement deficiency

Answer 35

CH50 assay

Question 36

CH50 assay measures

Answer 36

Intactness of the entire complement pathway

Question 37

Most common cause of an abnormal CH50 result

Answer 37

Delay in or improper transport of specimen to lab

Question 38

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

Answer 38

C4

Question 39

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

Answer 39

B cell and complement

Question 40

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

Answer 40

B cell

Question 41

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

Answer 41

C'

Question 42

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

Answer 42

T cell

Question 43

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

Answer 43

Neutrophil

Question 44

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

Answer 44

C'

Question 45

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

Answer 45

B cell

Question 46

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

Answer 46

Neutrophil

Question 47

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

Answer 47

Neutrophil

Question 48

B vs T vs C' vs neutrophil defect: Anergy

Answer 48

T cell

Question 49

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

Answer 49

B cell

Question 50

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

Answer 50

T cell

Question 51

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

Answer 51

Neutrophil

Question 52

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

Answer 52

Neutrophil

Question 53

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

Answer 53

T cell

Question 54

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

Answer 54

Neutrophil

Question 55

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

Answer 55

T cell

Question 56

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

Answer 56

Neutrophil

Question 57

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

Answer 57

T cell

Question 58

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

Answer 58

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

Question 59

Most common B cell defect

Answer 59

Selective IgA deficiency

Question 60

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

Answer 60

T