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Table 2 immunological features of LICS patients

From: New insights in phenotype and treatment of lung disease immuno-deficiency and chromosome breakage syndrome (LICS)

  Family 1 Family 2
A B C D E*
Immunological features
 IgA (g/L)a 2.2 (0.16–1.1) 0.6(0.16–1.1) 1.1(0.16–1.1) 1.0(0.16–1.1) 0.7 (0.19–2.2)
 IgG (g/L)a 11.1 (2.6–15.2) 7.5 (2.6–15.2) 11.6 (2.6–15.2) 5.3 (2.6–15.2) 8.2 (2.6–15.2)
 IgM (g/L)a 0.6 (0.1–1.2) 0.6 (0.1–1.2) 2.3 (0.1–1.2) 1.2 (0.1–1.2) 0.7 (0.1–1.2)
 IgE (g/L) 344 228 34 273 314
T cells
 CD4 + (109/L)b NA 2.42 (1.7–4.1) 1.12 (1.3–4.1) 28%c 1.38 (0.7–2.0)
 CD8 + (109/L)b NA 1.06 (0.7–1.8) 0.41 (0.5–1.6) 14%c 0.38 (0.5–1.4)
T cell proliferation to
 Mitogensd NA  =   =  NA ↓/= 
 Antigense NA  =  NA NA  = 
B cells (109/L)b NA 1.36 (0.8–2.2) 0.68 50%c 0.98 *
Antibody titers to
 Diphtheria NA  =  NA NA **
 Pneumococcal vaccination NA  =  NA NA **
Delayed type hypersensitivity testing
 Tetanus NA  =  NA NA **
 Candida NA  =  NA NA **
  1. = normal, NA not available, *at age 2, **not vaccinated
  2. aPresented as value (age-matched reference values of serum immunoglobulins)
  3. bPresented as value (age-related reference values for lymphocyte subpopulations p10–p90)
  4. cTotal cells 6 × 106, comment: in lymphoid fraction multiple B-cells, none monoclonal, in T-cell population no abnormalities
  5. dMitogens: Phytohemagglutinin, Concavalin A, Pokeweed
  6. eAntigens: Tetanus, candida