Table 3.

Immunological features

Immunological workup (at presentation)	P1 (2 years)	P2 (6 years)	P3 (21 months)	P4 (16 months)	P5 (3 years)	P6 (2 years)	P7 (5 years)	P8 (5 months)
IgG (g/L)	< 1.5	< 2.8	3.1	< 3.0	0.7	< 0.2	0.6	0.5
IgM (g/L)	< 0.18	< 0.25	< 0.25	3.63	< 0.17	0.6	1.6	0.18
IgA (g/L)	< 0.25	< 0.5	< 0.5	< 0.5	< 0.24	< 0.2	< 0.2	< 0.25
IgE (KU/L)	11.1	3.52	< 2	2	NA	< 2	< 5	< 2
CD3 (normal range)a	2.519 (2.2–4.1)	0.553 (1.7–1.9)	3788 (2.2–4.1)	0.827 (2.2–4.1)	1.561 (1.6–2.7)	2.777 (2.2–4.1)	2.403 (1.6–2.7)	2.500 (3.1–4.8)
CD4 (normal range)	1.842 (1.4–2.88)	0.399 (0.8–1.1)	2.892 (1.4–2.88)	0.528 (1.4–2.88	1.151 (1.0–1.7)	0.590 1.9 (1.4–2.88	0.940 1.2 (1.0–1.7)	1.520 (2.2–3.3)
CD8(normal range)	0.620 (0.8–1.8)	0.152 (0.7–1.0)	0.813 (0.8–1.8)	0.268 (0.8–1.8)	0.388 (0.61–1.0)	2.123 (0.8–1.8)	1.350 (0.61–1.0)	0.930 (1.1–1.7)
CD4/CD8	2.9	2.6	3.6	3.2	3.5	0.3	0.7	1.63
CD19 (normal range)	1.809 (0.7–1.6)	0.041 (0.4–0.8)	1.142 (0.7–1.6)	1.285 (0.7–1.6)	0.189 (0.4–0.8)	0.051 (0.7–1.6)	0.079 (0.4–0.8)	1.670 (1.1–1.9)
NK cell (normal range)	0.079 (0.2–0.6)	0.098 (0.2–0.4)	0.079 (0.2–0.6)	0.042 (0.2–0.6)	0.090 (0.2–0.48)	0.037 (0.2–0.6)	0.312 (0.2–0.48)	0.490 (0.3–0.7)
CD3 CD4 RA %	ND	ND	60	52	65	ND	ND	78
CD3 CD4 RO %	ND	ND	10	14	49	ND	ND	18
PHA	73.730 (53% of the controls)b	84,550 (70 %)	78,626 (42%)	16,613 (28%)	25,618 (25%)	13,390 (12%)	39,976 (29%)	ND

ND not done

^aNormal range for age

^bResult in percentage compared to controls