Answers 2024/2025
transient hypogammaglobulinemia of infancy
X linked Bruton's agammaglobulinemia
IgA deficiency
common variable immunodeficiency (CVI) - ANSWERSlist the primary deficiencies of the B cell system.
DiGeorge anomaly
purine-nucleoside phosphorylase (PNP) deficiency - ANSWERSlist the cellular immunity deficiencies.
severe combined immunodeficiency (SCID)
wiskott-aldrich syndrome (WAS)
ataxia-telangiectasia (AT) - ANSWERSlist the combined deficiencies of cellular and humoral immunity.
oxidative metabolism disorders (chronic granulomatous dz and myeloperoxidase deficiency)
leukocyte adhesion molecule deficiency - ANSWERSlist the two types of neutrophil function defects.
secondary immune deficiency - ANSWERSwhat is an abnormality due to another disorder, infection, or
treatment?
malignancies: hodgkins dz and lymphoma, leukemia, myeloma, macroglobulinemia
infections: bacterial, viral, protozoan, helminthic, fungal; most prominent among these is AIDS
other conditions: burns, diabetes, malnutrition, surgical trauma, renal malfunction, aging, alcoholic
cirrhosis - ANSWERSlist the secondary immune deficiencies.
transient hypogammaglobulinemia of infancy - ANSWERSwhat is the deficiency?
, prolonged low Ig levels, children have problems with infections, and delay in Ig levels attributed to poor
T cell help
X linked brutons agammaglobulinemia - ANSWERSwhat is the deficiency?
development of B cell blocked at pro-B cell to pre-B cell, deficient in tyrosine kinase, small/absent and
abnormal lymphoid tissue, persitent beyond 2 years of age, treat with Ig preparation
IgA deficiency - ANSWERSwhat is the deficiency?
most common congenital immunodeficiency, most asymptomatic, anti-antibodies produced by some
(cause anaphylaxsis following transfusion), replacement therapy not useful
common variable immunodeficiency (CVI) - ANSWERSwhat is the deficiency?
normal # of B cells, cannot differentiate into Ig producing cells, usually in 20s and 30s, manifestations
(malabsorption/diarrhea, malignancies, autoimmune disorders), IgG & IgA deficient, recurrent infections,
treat with Ig preparations and antibiotics
DiGeorge anomaly - ANSWERSwhat is the deficiency?
missing portion of chromosome 22, embryonic development defect in thymus and other organs,
insufficient # of mature T cells, treat with fetal thymus and BM transplant and thymic hormones
purine-nucleoside phosphorylase - ANSWERSwhat is the deficiency?
defect in enzyme involved in purine metabolism, # of T cells decrease as toxic purine metabolite
accumulates, humoral immunity OK
severe combined immunodeficiency (SCID) - ANSWERSwhat is the deficiency?