Wiskott-Aldrich Syndrome Flashcards
1
Q
Genetic or acquired?
A
Genetic: x-linked recessive syndrome
2
Q
typified by
A
decrease in T cells
3
Q
how it effects cells
A
cytoskeletal component called WAS-protein (WASP) not produced. function not entirely understood.
4
Q
characterized by
A
thrombocytopenia, small platelets, platelet dysfunction
Eczema, susceptibility to infections
5
Q
Ab situation
A
Low IgM; IgG normal; IgA and IgE elevated
6
Q
Infants typically present with
A
prolonged bleeding at circumcision site
bloody diarrhea
excessive bruising
7
Q
Where is WASP expression found?
A
it’s limited to hematopoietic lineages
8
Q
Patients with this are at risk for
A
cancer and autoimmune diseases
