Immunodeficiency diseases

Question 1

X linked agammaglobulinemia

Answer 1

-Male infants >6 months
- recurrent BACTERIAL infections like h.infuenzae, strep staph
- unaffected immunity against viral/fungal infections (except coxsackie and ECHO viruses)
-FEATURES: low plasma b cells, therefore no IGs in tissues
failure of maturation of b cells
-absent/ poorly defined germinal centers

Question 2

Isolated igA deficiency

Answer 2

-#1 inherited b cell defect
-asymptomatic with occasional diarrhea and anaphylaxis t transfused blood products
-igA plasma cells fail to develop

Question 3

Common variable immunodeficiency

Answer 3

-Recurrent BACTERIAL infections
-Hypogammaglobulinemia why? due to failure of development of terminal b cells, decreasing the number of plasma cells
-

Question 4

DiGeorge syndrome

Answer 4

-congenital aberration of 3rd/4th branchial arches that house the thymus and parathyroid gland embryogenically
cause lack of T cells and hypocalcemia
Recurrent VIRAL and FUNGAL infections
-CATCH 22
Cardiac defects
Abnormal facies
Thymic hypoplasia
Cleft palate
Hypocalcemia- causing tetany
22- microdeletion n chromosome 22

Question 5

SCID

Answer 5

Sever Combined Immunodeficiency