Table 1. Immunological evaluation of the... | Rockefeller University Press

Table 1.

Immunological evaluation of the CTLA4^S172P/S172P patient

Parameters	Before abatacept (Age: 7 years)	On abatacept (At 9 mo of therapy)	Reference values (5–10 years)
Leukocyte count (/μl)	3,100 (↓)	9,000	4,000–10,000
Absolute lymphocyte count (/μl)	1,200 (↓)	4,300	1,803–5,636
Absolute neutrophil count (/μl)	1,100 (↓)	4,000	2,000–7,000
Absolute eosinophil count (/μl)	110	210	20–500
Hemoglobin (g/dl)	11.2	14	11–16
Platelet (x10³/μl)	6 (↓)	315	150–450
IgG (mg/dl)	677 (↓)	On IgRT	764–2,134
IgA (mg/dl)	146	89	78–383
IgM (mg/dl)	74	42 (↓)	69–387
IgE (IU/ml)	3	7	<200
Specific antibody titers
Anti-HBs IgG (mIU/ml)	<0.2 (−)	On IgRT	<10 negative
Anti-HBs IgG (mIU/ml)	<0.2 (−)	On IgRT	>= 10 positive
Anti-measles IgG (IU/L)	0.82 (−)	On IgRT	<0.9 negative
			0.9–1.1 borderline
			>1.1 positive
Anti-mumps IgG (U/ml)	1.51 (+)	On IgRT	<0.9 negative
			0.9–1.1 borderline
			>1.1 positive
Anti-varicella zoster IgG (S/CO)	0.57 (−)	On IgRT	<0.9 negative
			0.9–1.1 borderline
			>1.1 positive
Lymphocyte subsets
CD3⁺ T cells (%)	77	66	55–86
CD3⁺ count (/mm³)	925 (↓)	2,824	971–3,685
CD3⁺ CD4⁺ (%)	40	39	23–49
CD3⁺ CD4⁺ count (/mm³)	480	1,670	445–1,918
CD3⁺ CD8⁺ (%)	28	20	17–46
CD3⁺ CD8⁺ count (/mm³)	336 (↓)	856	379–2,084
CD19⁺ B cells (%)	16	27 (↑)	7–20
CD19⁺ count (/mm³)	192	1,155 (↑)	122–755
CD16 ⁺ 56⁺ NK cells (%)	3 (↓)	5	4–29
CD16 ⁺ 56⁺ NK cell count (/mm³)	38 (↓)	201	105–1,107
CD19⁺CD27⁻IgD⁺ B cells (%)	89.1 (↑)	88 (↑)	45–85
CD19⁺CD27⁺IgD⁺ B cells (%)	2.8 (↓)	2.5 (↓)	4–24
CD19⁺CD27⁺IgD⁻ B cells (%)	1.1 (↓)	2.6 (↓)	7–31
CD21^lowCD38^low activated B (%)	30.3 (↑)	9.8	2–14
CD3⁺ TCR^α/β cells (%)	77	85	68–96
CD3⁺ TCR^γ/δ cells (%)	12	11	5–30
CD4⁺CD45RA⁺CD31⁺ T cells (%)	27.8 (↓)	53.6	39–66
CD4⁺CD45RA⁺CCR7⁺ T cells (%)	26.6 (↓)	57.6	38–75
CD4⁺CD45RA⁻CCR7⁺ T cells (%)	27.1	35.1	14–49
CD4⁺CD45RA⁻CCR7⁻ T cells (%)	38.4 (↑)	6.1	2–16
CD4⁺CD45RA⁺CCR7⁻ T cells (%)	7.7	1.2	0–44
CD8⁺CD45RA⁺CCR7⁺ T cells (%)	28.1	77.9 (↑)	17–62
CD8⁺CD45RA⁻CCR7⁺ T cells (%)	5.3	10.5 (↑)	0–8
CD8⁺CD45RA⁻CCR7⁻ T cells (%)	37 (↑)	5.5	2–33
CD8⁺CD45RA⁺CCR7⁻ T cells (%)	29.6	6.1	14–61

Parameters	Before abatacept (Age: 7 years)	On abatacept (At 9 mo of therapy)	Reference values (5–10 years)
Leukocyte count (/μl)	3,100 (↓)	9,000	4,000–10,000
Absolute lymphocyte count (/μl)	1,200 (↓)	4,300	1,803–5,636
Absolute neutrophil count (/μl)	1,100 (↓)	4,000	2,000–7,000
Absolute eosinophil count (/μl)	110	210	20–500
Hemoglobin (g/dl)	11.2	14	11–16
Platelet (x10³/μl)	6 (↓)	315	150–450
IgG (mg/dl)	677 (↓)	On IgRT	764–2,134
IgA (mg/dl)	146	89	78–383
IgM (mg/dl)	74	42 (↓)	69–387
IgE (IU/ml)	3	7	<200
Specific antibody titers
Anti-HBs IgG (mIU/ml)	<0.2 (−)	On IgRT	<10 negative
Anti-HBs IgG (mIU/ml)	<0.2 (−)	On IgRT	>= 10 positive
Anti-measles IgG (IU/L)	0.82 (−)	On IgRT	<0.9 negative
			0.9–1.1 borderline
			>1.1 positive
Anti-mumps IgG (U/ml)	1.51 (+)	On IgRT	<0.9 negative
			0.9–1.1 borderline
			>1.1 positive
Anti-varicella zoster IgG (S/CO)	0.57 (−)	On IgRT	<0.9 negative
			0.9–1.1 borderline
			>1.1 positive
Lymphocyte subsets
CD3⁺ T cells (%)	77	66	55–86
CD3⁺ count (/mm³)	925 (↓)	2,824	971–3,685
CD3⁺ CD4⁺ (%)	40	39	23–49
CD3⁺ CD4⁺ count (/mm³)	480	1,670	445–1,918
CD3⁺ CD8⁺ (%)	28	20	17–46
CD3⁺ CD8⁺ count (/mm³)	336 (↓)	856	379–2,084
CD19⁺ B cells (%)	16	27 (↑)	7–20
CD19⁺ count (/mm³)	192	1,155 (↑)	122–755
CD16 ⁺ 56⁺ NK cells (%)	3 (↓)	5	4–29
CD16 ⁺ 56⁺ NK cell count (/mm³)	38 (↓)	201	105–1,107
CD19⁺CD27⁻IgD⁺ B cells (%)	89.1 (↑)	88 (↑)	45–85
CD19⁺CD27⁺IgD⁺ B cells (%)	2.8 (↓)	2.5 (↓)	4–24
CD19⁺CD27⁺IgD⁻ B cells (%)	1.1 (↓)	2.6 (↓)	7–31
CD21^lowCD38^low activated B (%)	30.3 (↑)	9.8	2–14
CD3⁺ TCR^α/β cells (%)	77	85	68–96
CD3⁺ TCR^γ/δ cells (%)	12	11	5–30
CD4⁺CD45RA⁺CD31⁺ T cells (%)	27.8 (↓)	53.6	39–66
CD4⁺CD45RA⁺CCR7⁺ T cells (%)	26.6 (↓)	57.6	38–75
CD4⁺CD45RA⁻CCR7⁺ T cells (%)	27.1	35.1	14–49
CD4⁺CD45RA⁻CCR7⁻ T cells (%)	38.4 (↑)	6.1	2–16
CD4⁺CD45RA⁺CCR7⁻ T cells (%)	7.7	1.2	0–44
CD8⁺CD45RA⁺CCR7⁺ T cells (%)	28.1	77.9 (↑)	17–62
CD8⁺CD45RA⁻CCR7⁺ T cells (%)	5.3	10.5 (↑)	0–8
CD8⁺CD45RA⁻CCR7⁻ T cells (%)	37 (↑)	5.5	2–33
CD8⁺CD45RA⁺CCR7⁻ T cells (%)	29.6	6.1	14–61

IgRT, immunoglobulin replacement therapy; CD19⁺CD27⁻IgD⁺, naïve mature B cells; CD19⁺CD21^lowCD38^low, autoreactive B cells; CD19⁺CD27⁺IgD⁺, non–class-switched memory B cells; CD19⁺CD27⁺IgD⁻, class-switched memory B cells; CD4⁺CD45RA⁺CCR7⁺, CD4⁺ naïve T cells; CD8⁺CD45RA⁺CCR7⁺, CD8⁺ naïve T cells; CD4⁺CD45RA⁺CD31⁺, recent thymic emigrants; CD4⁺CD45RA⁻CCR7⁺, central memory CD4⁺ T cells; CD4⁺CD45RA⁻CCR7⁻, effector memory CD4⁺ T cells; CD4⁺CD45RA⁺CCR7⁻, terminally differentiated effector memory CD4⁺ T cells; CD8⁺CD45RA⁻CCR7⁺, central memory CD8⁺ T cells; CD8⁺CD45RA⁻CCR7⁻, effector memory CD8⁺ T cells; CD8⁺CD45RA⁺CCR7^–, terminally differentiated effector memory CD8⁺ T cells. Abnormal values are indicated boldly with upward (↑) or downward (↓) arrows in parentheses.

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