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