Table 1

Median lymphocyte numbers and immunoglobulin levels in 22q11.2 deletion patients with severe or recurrent infection

Patient (age)Follow up (y)InfectionCD3 (cells/μl)CD4 (cells/μl)CD8 (cells/μl)CD19 (cells/μl)IgM (g/l)IgA (g/l)IgG (g/l)IgG subclass (g/l)Sp AbsTreatment
URTI, upper respiratory tract infection; LRTI, lower respiratory tract infection; IVIG, intravenous immunoglobulin.
*Recurrent infection which resolved with increasing age.
Klebsiella oxytoca septicaemia.
Pseudomonas aeruginosa pneumonia.
*1 (6 y) 6URTI, OM13218115333911.0 1.51 5.97NLow PPSNil
    2 (7 y) 6Pneumonia, URTI12937254009451.02<0.0713.3High IgG1NCo-trimoxazole
    3 (12 y) 4Recurrent pneumonia378258109920.19<0.07 2.2Low IgG2Low Tet, PPSIVIG
    4 (9 y) 8OM, otorrhoea0.68 2.3116.8NNNil
    5 (12 y) 7Pneumonia, URTI, OM5602952743060.28 0.57 7.85NNCo-trimoxazole
    6 (22 y)10URTI5052432971560.66 1.111.4NLow PPSCo-trimoxazole
    7 (4 y) 2URTI, otorrhoea2163126080811470.33 0.39 5.7NLow PPSCo-trimoxazole
    8 (12 y) 1OM, mastoiditis6433552443130.6 0.5813.0NNNil
    9 (8 y) 8URTI, endocarditis161510525675500.46 1.54 7.95NLow Tet, PPSCo-trimoxazole
    10 (0.5 y) 0.5Septicaemia272519589195190.52 0.22 2.8IVIG
    11 (0.5 y) 0.5Pneumonia‡10488162653670.53 0.19 4.02NCo-trimoxazole, IVIG
    12 (5 y) 4URTI0.5 0.44 9.0NNCo-trimoxazole
*13 (9 y) 7URTI12296135934130.49 1.59 9.48NLow PPSNil
    14 (7 y) 4OM3000124818715960.59 0.53 8.98Low IgG2Low PPSCo-trimoxazole
    15 (4 y) 4LRTI15429715507620.49 0.27 5.25NNCo-trimoxazole
    16 (13 y) 6LRTI Bronchiectasis1264122410105810.86<0.0714.9Low IgG2Low Tet, PPSCo-trimoxazole
*17 (17 y) 2Pneumonia, sinusitis7784393102331.2 1.87 8.61NNNil
    18 (1 y) 1Septicaemia8506512273260.3<0.07 5.0NNNil
    19 (12 y) 2Pneumonias12246294372970.77 1.710.6NNil
    20 (5 y) 5URTI/LRTI7483393516190.31 0.37 4.42NLow PPSCo-trimoxazole
    21 (6 y) 6URTI, otorrhoea7993823097650.89 0.73 7.4NLow PPSCo-trimoxazole
*22 (4 y) 2URTI, pneumonia6263133023830.72 0.32 9.6NNCo-trimoxazole
    23 (9 y) 9URTI15828315829850.54 1.8312.1NNNil
    24 (8 y) 2URTI, pneumonia8213973165580.37 0.93 7.15Low IgG2Low PPSIVIG, co-trimoxazole
    25 (5 y) 1URTI8523555033260.75 0.7910.2Low IgG1NNil
    26 (8 y) 1Pneumonia, otorrhoea, mastoiditis8965123982700.55 0.66 4.35Low IgG1NNil