Primary Immunodeficiency Flashcards
1
Q
Most Common Immunodeficiency
A
Selective IgA deficiency
2
Q
Presentation of Bc vs. SCID Deficiencies
A
After 6 months vs. immediately
3
Q
X-Linked Agammaglobulinemia
A
Defect in Btk, so absence in all mature B cells
4
Q
WAS gene
A
Primarily affects platelets, DCs, and macs
5
Q
X-Linked Severe Combined Immunodeficiency
A
Defect in common gamma chain for Ts which wipes out whole adaptive immune system bc Bs are useless without Ts
6
Q
DiGeorge Syndrome
A
SCID from thymic aplasia
7
Q
Hyper IgM Syndrome
A
Downstream Bc effect, deficiency in AID or CD40L which are required for class switch
8
Q
Bc vs. SCID Dominant Infections
A
Respiratory bacterial vs. Viral
9
Q
Bc vs. SCID Treatments
A
IVIG or BMT (cure) vs. BMT or stem cell/gene therapy
