Table 3.

Lymphocyte, memory B-cell populations, and serology

a)
	Patient II-1	Patient III-1	Reference range
Lymphocyte count (cells × 109/L)	1.9	1.1	1.2–2.7
CD3+ (%)	86	78	49–84
CD3+ (cells × 109/L)	1.63	0.86	0.6–2.99
CD19+ (%)	6	10	7–27
CD19+ (cells × 109/L)	0.11	0.11	0.11–0.7
CD4+ (%)	43	55	28–63
CD4+ (cells × 109/L)	0.82	0.6	0.44–2.16
CD8+ (%)	40	20	10–40
CD8+ (cells × 109/L)	0.76	0.22	0.13–1.31
CD16+CD56+ (%)	7	11	4–25
CD16+CD56+ (cells × 109/L)	0.13	0.12	0.1–0.64
CD4+:CD8+ ratio	1.08	2.75	0.6–5
b)
	Patient II-1	Patient III-1	Reference rangea
Total CD19+ B cells (% of lymphocytes)	4.9	7.8	5–26
Total CD19+ B cells (cells × 109/L)	0.093	0.086	58–558
CD19+ 27+ memory B cells (% of B cells)	41	11	7–48
CD19+ 27+ memory B cells (cells × 109/L)	0.038	0.0097	0.013–0.148
CD19+ 27+IgM−IgD− switched memory B cells (% of B cells)	35	5.8	3–23
CD19+ 27+IgM−IgD−switched memory B cells (cells × 109/L)	0.032	0.0050	0.004–0.066
CD19+ 27−IgM+IgD+naïve B cells (% of B cells)	55	87	29–93
c)
	Patient II-1	Patient III-1	Reference range
Diphtheria IgG (IU/mL)	0.26	n.d.	> 0.1
Haemophilus influenzae IgG	0.47	n.d.	N/A
Pneumococcal IgG (mcg/mL)	7.5	16.9	> 39
Tetanus IgG (IU/mL)	2.59	0.84	> 0.16

IgG immunoglobulin G. ^aBased on published data. Abnormal results are shown in bold