Wiskott Aldrich syndrome

Question 1

Wiskott Aldrich syndrome etiology

Answer 1

X-linked recessive , WASP gene mutation

• No T cells → increase risk for viral and fungal
  infection

Question 2

WATER (mnemonic)

Answer 2

*Wiskott-Aldrich , *Thrombocytopenia ,
*Eczema/Atopic dermatitis ,
*Recurrent infections

Question 3

labs

Answer 3

IgM Low , IgG N/Low ;
IgA HIGH, IgE HIGH

Question 4

Treatment

Answer 4

: - Definitive: HSCT /BMT - IVIG once a month - Killed vaccine - Eczema treatment : skin lubricant, topical
antibiotics

Question 5

first sign to appear in WAS is?

Answer 5

bloody diarrhea