Evaluation Of Suspected Immunodeficiency Flashcards
Most cost-effective screening tests for immunodeficiency
CBC, ESR
Primary immunodeficiency: HypoCa, unusual facies and ears, heart disease
DiGeorge anomaly
Primary immunodeficiency: Delayed umbilical cord detachment, leukocytosis, recurrent infections
Leukocyte adhesion deficiency
Primary immunodeficiency: Persistent thrush, failure to thrive, pneumonia, diarrhea
Severe combined immunodeficiency
Primary immunodeficiency: Bloody stools, draining ears, atopic eczema
Wiskott-Aldrich syndrome
Primary immunodeficiency: P. jiroveci pneumonia, neutropenia, recurrent infections
X-linked hyper-IgM syndrome
Primary immunodeficiency: Severe progressive infectious mononucleosis
X-linked lymphoproliferative syndrome
Primary immunodeficiency: Recurrent staphylococcal abscesses, staph pneumonia with pneumatocele formation, coarse facial facies, pruritic dermatitis
Hyper IgE syndrome
Primary immunodeficiency: Persistent thrush, nail dystrophy, endocrinopathies
Chronic mucocutaneous candidiasis
Primary immunodeficiency: Short stature, fine hair, severe varicella
Cartilage hair hypoplasia with short-limbed dearfism
Primary immunodeficiency: Oculocutaneous albinism, recurrent infection
Chediak-Higashi syndrome
Primary immunodeficiency: Abscesses, suppurative lymphadenopathy, antral outlet obstruction, pneumonia, osteomyelitis
Chronic granulomatous disease
Primary immunodeficiency: Progressive dermatomyositis eith chronic enterovirus encephalitis
X-linked agammaglobulinemia
Primary immunodeficiency: Sinopulmonary infections, neurologic deterioration, telangiectasia
Ataxia-telangiectasia
Primary immunodeficiency: Recurrent neisserial meningitis
C6, C7 or C8 deficiency
Primary immunodeficiency: Sinopulmonary infections, splenomegaly, autoimmunity, malabsorption
Common variable immunodeficiency
A normal absolute lymphocyte count (ALC) result rules out against
T cell defect
A normal ANC rules out against
1) Congenital or acquired neutropenia 2) Both forms of leukocyte adhesion deficiency
Normal platelet count excludes
Wiskott-Aldrich syndrome
Evaluation of suspected immunodeficiency should be initiated in the presence of
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
Absence of Howell-Jolly bodies rules out
Asplenia
ESR result in chronic bacterial infection
Normal
Normal ESR result indicates that this infection is unlikely
Fungal
Screening tests for B cell defects
IgA measurement, if abnormal, IgM and IgG measurment
Candida albicans intradermal skin test is a screening test for
T cell defects
Respiratory burst assay is a screeningg test for
Phagocytic cell defect
CH50 is a screening test for
Complement deficiency
Most common B cell defect
IgA deficiency
Measurement of isohemagglutinins (anti-A and anti-B) measures predominantly what type of Ig
IgM
Most cost-effective test of T cell function
Candida skin test
Positive Candida skin test
Erythema and induration >/ 10mm at 48hrs
Killing defect of phagocytic cells should be suspected of a patient has
1) Recurrent staph abscesses 2) Recurrent G- infections
Most reliable and useful test for phagocytic cells
Flow cytometry after respiratory burst using rhodamine dye, which has replaced NBT dye test
Early diagnosis of LAD is of crucial importance because
Stem cell transplantation is life-saving
Most effective screening test for complement deficiency
CH50 assay
CH50 assay measures
Intactness of the entire complement pathway
Most common cause of an abnormal CH50 result
Delay in or improper transport of specimen to lab
Measurement of this complement factor can be helpful in assessing suspected hereditary angioedema
C4
B vs T vs C’ vs neutrophil defect: Recurrent pyogenic infections with extracellular encapsulated organisms
B cell and complement
B vs T vs C’ vs neutrophil defect: Otitis, sinusitis, recurrent pneumonia, bronchiectasis, and conjunctivitis
B cell
B vs T vs C’ vs neutrophil defect: Recurrent N meningitidis infection
C’
B vs T vs C’ vs neutrophil defect: Recurrent infections with less virulent or opportunistic organisms
T cell
B vs T vs C’ vs neutrophil defect: Recurrent dermatologic infections
Neutrophil
B vs T vs C’ vs neutrophil defect: Increased incidence of autoimmune disease
C’
B vs T vs C’ vs neutrophil defect: Diarrhea common
B cell
B vs T vs C’ vs neutrophil defect: Subcu, lymph node, lung, and liver abscesses
Neutrophil
B vs T vs C’ vs neutrophil defect: Pulmonary infections including abscess and pneumatocele
Neutrophil
B vs T vs C’ vs neutrophil defect: Anergy
T cell
B vs T vs C’ vs neutrophil defect: Minimal growth retardation
B cell
B vs T vs C’ vs neutrophil defect: Susceptible to graft vs host disease from non irradiated blood or from maternal engraftment
T cell
B vs T vs C’ vs neutrophil defect: Bone and joint infection common
Neutrophil
B vs T vs C’ vs neutrophil defect: Delayed separation of umbilical cord
Neutrophil
B vs T vs C’ vs neutrophil defect: Fatal reactions from live virus or BCG vaccination
T cell
B vs T vs C’ vs neutrophil defect: Absence of pud at site/s of infection
Neutrophil
B vs T vs C’ vs neutrophil defect: High incidence of malignancy
T cell
B vs T vs C’ vs neutrophil defect: Poor wound healing
Neutrophil
B vs T vs C’ vs neutrophil defect: Poor survival beyond infancy or early childhood
T cell
Evaluation of immune function should be initiated in those rare infants or children who
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
Most common B cell defect
Selective IgA deficiency
T/F Antibody titers are not interpretable after the patient has received a blood transfusion
T
