B Cell Deficiencies Flashcards
Deficiency tyrosine kinase that block B cell maturation
Bruton X linked agammaglobulinemia (Ig replacement)
Low Ig, no circulating B cells
Bruton X linked agammaglobulinemia (Ig replacement)
Deficiency of CD40 L on activated T cell
X linked hyper IgM syndrome (Ig replacement)
High serum titers of IgM, susceptible to extracellular bacteria
X linked hyper IgM syndrome (Ig replacement)
Most common B cell deficiency
Selective IgA deficiency
Repeated sinopulmonary and GI infxn
Selective IgA deficiency
Ig decrease over time
Common variable immunodeficiency (antibiotics)
Delayed onset of maturation of normal IgG
Transient agammaglobulinia (antibiotics)
Spontaneous resolution, diagnosed in infants
Transient agammaglobulinemia (antibiotics)
DiGeorge syndrome
Congenital thymic aplasia
Only disease that is T cell specific; facial abnormalities, hypoparathryoidism, cardiac malformation
DiGeorge syndrome
CATCH22 syndrome
Cardiac abnormalities, abnormal facies, thymic hypoplasia, cleft palate, hypocalcemia
Defect in cystoskeletal glycoprotein
Wiskott adlrich
Defective response in bacterial polysaccharide and depressed IgM , gradual loss of humoral and immune response, thrombocytopenia and ECZYMA
Wiskot aldrich
Adenosine deaminase deficiency
Severe combined ID
Prone to opportunistic infection, low levels of circulating lymphocytes, chronic diarrhea, skin and mout infections
Severe combined immunodeficiecy
Deficiency in NADPH oxidase
Chronic granulomatous disease
Recurrent infection with Catalase positive organisms
Chronic granulomtous disease
Absence of CD 18
Leukocyte adhesion defect
Leukocytosis, failure to form pus, delay in umbilical cord sloughing
Leukocyte adhesion defect
Granule structural defect
Chediak Higashi syndrome
Recurrent bacterial infection , partial albinism
Chediak Higashi syndrome
Deficiency in TNF gamna
Jobs syndrome
Coarse facies, cold abscess, Inc IgE
Jobs syndrome
