Primary Immunodeficiencies AB Flashcards
(3 cards)
1
Q
What are the features of Wiskott-Aldrich syndrome?
A
Combined B and T cell dysfunction
- Recurrent infections (e.g. chest)
- Eczema
- Thrombocytopaenia
- Low IgM levels
2
Q
What is the genetic abnormality in Wiskott-Aldrich syndrome?
A
X-linked recessive
WASP gene
3
Q
What is the treatment of hereditary angioedema?
A
Acute: C1-inhibitor concentrate (or FFP if not available)
Prophylaxis: Anabolic steroids e.g. Danazole
