Table 2.

Clinical features and laboratory investigations

Clinical symptoms	Recurrent infections Bronchiectasis
Family history of hypogammaglobulinemia	Daughter with CVID
Hb (130–190 g/l)	118
Neutrophils (1.9–7.5 × E + 9/l)	1.9
Platelets (150–400× E + 9/l)	208
IgG (7–14 g/l)	3.5
IgA (0.8–4 g/l)	< 0.07
IgM (0.4–2.5 g/l)	0.4
Autoantibodies	Nil
Immunophenotype	B cells present
Switched memory B cells (CD19+ CD27+ , IgD−: NR 5–21)	1.8
Tetanus pre/post (IU/ml)	0.64/0.52
Diphtheria pre/post (IU/ml)	0.03/0.08
HIB pre/post µg/ml	0.16/0.37
Pneumovax® pre/post* (serotypes > 1.3 µg/ml/23)	0/0
Vaccine durability	Not done
Sequence variations	WES: no causative mutation
Relevant histology	Nil
T cell responses	Normal lectin responses Normal responses to tetanus, diphtheria and candida
Diagnosis	CVID (Ameratunga et al. criteria)
Treatment	IVIG/SCIG Bariatric surgery
Clinical outcome	Well