Sunday, 31 October 2010

MRCP revision battle 38.4: Combined B and T cell disorders

So having skated through B cell disorders and T cell disorders its on to combined disorders, the main 3 of which may be recalled as SCID WAS ATAXIC.

1. Severe Combined Immundeficiency
  • autosomal recessive
  • sometimes due to lack of adenosine deaminase

2. Wiskott-Aldrich Syndrome
  • x-linked recessive
  • characterised by recurrent infections, eczema and thrombocytopenia
  • associated with an increased risk of maligancy

3. Ataxic telangiectasia
  • autosomal recessive
  • cerebellar ataxia and telangiectasia
  • increased risk of malignancy
  • low IgE and IgA

Lets now move back a bit more mainstream with thrombotic thrombocytopenic purpura...