Table 2 Immunological characteristics of... | American Society of Hematology

Table 2

Immunological characteristics of XLF-deficient patients

Patient	Lymphocytes	T cells	CD4+ T cells	CD8+ T cells	B cells	NK cells	Age at evaluation
P1⁷	0.8(1.4-3.3)	0.6(1.0-2.2)	0.4(0.5-1.3)	0.2(0.3-0.9)	0(0.1-0.6)		14 y
P2⁷	1.2(2.3-5.4)	0.7(1.4-3.7)	0.2(0.7-1.5)	0.6 (0.4-1.1)	0.08(0.4-1.4)	0.3 (0.09-0.9)	2 y
P3⁷	1.6 (1.4-3.3)	0.9(1.0-2.2)	0.5 (0.5-1.3)	0.4 (0.3-0.9)	0(0.1-0.6)	0.4 (0.04-0.7)	13 y
P4⁷	2.6 (2.3-5.4)	0.6(1.4-3.7)	0.3(0.7-1.5)	0.08(0.4-1.1)	0.2(0.4-1.4)	1.1 (0.09-0.9)	2 y
P5⁷	1.1(1.9-3.7)	0.7(1.2-2.6)	0.3(0.5-1.3)	0.3 (0.3-0.9)	0.04(0.1-0.6)	0.2 (0.08-0.7)	7 y
P6		0.6(2.3-5.4)			0.08(0.4-1.4)		6 y
P7		1.3(1.9-3.7)			0.04(0.1-0.6)		2 y
P8		0.2(1.4-3.3)			0(0.1-0.6)		11 y
XLF1	1.4(1.7-6.9)	1.1 (0.9-4.5)	0.6 (0.5-2.4)	0.4 (0.3-1.6)	0.06(0.2-2.1)	0.2 (0.1-1.0)	4 y
XLF5	0.7(1.1-5.9)	0.5(0.7-4.2)	0.3 (0.3-2.0)	0.2(0.3-1.8)	0(0.2-1.6)		8 y
XLF6-1	1.5(2.6-10.4)	0.2(1.6-6.7)	0(1.0-4.6)	0.1(0.4-2.1)	0.04(0.6-2.7)	0(0.2-1.2)	11 mo
XLF6-2	0.9(1.1-5.9)	0.9 (0.7-4.2)	0.3 (0.3-2.0)	0.4 (0.3-1.8)	0.01(0.2-1.6)	0.1 (0.09-0.9)	8 y

Patient	Lymphocytes	T cells	CD4+ T cells	CD8+ T cells	B cells	NK cells	Age at evaluation
P1⁷	0.8(1.4-3.3)	0.6(1.0-2.2)	0.4(0.5-1.3)	0.2(0.3-0.9)	0(0.1-0.6)		14 y
P2⁷	1.2(2.3-5.4)	0.7(1.4-3.7)	0.2(0.7-1.5)	0.6 (0.4-1.1)	0.08(0.4-1.4)	0.3 (0.09-0.9)	2 y
P3⁷	1.6 (1.4-3.3)	0.9(1.0-2.2)	0.5 (0.5-1.3)	0.4 (0.3-0.9)	0(0.1-0.6)	0.4 (0.04-0.7)	13 y
P4⁷	2.6 (2.3-5.4)	0.6(1.4-3.7)	0.3(0.7-1.5)	0.08(0.4-1.1)	0.2(0.4-1.4)	1.1 (0.09-0.9)	2 y
P5⁷	1.1(1.9-3.7)	0.7(1.2-2.6)	0.3(0.5-1.3)	0.3 (0.3-0.9)	0.04(0.1-0.6)	0.2 (0.08-0.7)	7 y
P6		0.6(2.3-5.4)			0.08(0.4-1.4)		6 y
P7		1.3(1.9-3.7)			0.04(0.1-0.6)		2 y
P8		0.2(1.4-3.3)			0(0.1-0.6)		11 y
XLF1	1.4(1.7-6.9)	1.1 (0.9-4.5)	0.6 (0.5-2.4)	0.4 (0.3-1.6)	0.06(0.2-2.1)	0.2 (0.1-1.0)	4 y
XLF5	0.7(1.1-5.9)	0.5(0.7-4.2)	0.3 (0.3-2.0)	0.2(0.3-1.8)	0(0.2-1.6)		8 y
XLF6-1	1.5(2.6-10.4)	0.2(1.6-6.7)	0(1.0-4.6)	0.1(0.4-2.1)	0.04(0.6-2.7)	0(0.2-1.2)	11 mo
XLF6-2	0.9(1.1-5.9)	0.9 (0.7-4.2)	0.3 (0.3-2.0)	0.4 (0.3-1.8)	0.01(0.2-1.6)	0.1 (0.09-0.9)	8 y

Absolute counts (×10⁹/L). The age-matched controls values are shown in italics in parentheses.^57,58 Reduced values are indicated in bold.