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...