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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