Wiskott-Aldrich

Question 1

Inheritance mode of WA

Answer 1

X-linked recessive

Question 2

Triad of sx in WA

Answer 2

Microthrombocytopenia, recurrent severe pyogenic infections, and mild to severe atopic dermatitis (often with serosanguinous or hemorrhagic crust due to platelet dysfunction)

Question 3

Prognosis in WA

Answer 3

Poor 2/2 fatal infections vs. hemorrhage vs. lymphoreticular malignancies

Question 4

Tx of WA

Answer 4

bone marrow transplant

Question 5

Genetic etiology of WA

Answer 5

mutation in WAS protein that polymerizes the actin cytoskeleton to allow for cell movement/immune synapses/dendrite formation