Table 3.

Immunoglobulin levels and affected cell lineage in peripheral blood prior to HSCT

Patient no	Affected cell lineage (normal range)							Immunoglobulins		Time before HSCT (months)
	CD19 + , cells × 106/L (100–500)	NK cells × 106 /L (100–400)	CD3 +  T- cells × 106/L (800–2400)	CD4 +  T- cells × 106/L (500–1400)	CD8 +  T-cells × 106/L (200–2000)	Monocytes  × 109/L (0.2–0.8)	Neutrophils  × 109/L (1.5–7.3)	IgG g/L (6.9–15.7)	IgG2 g/L (1.69–15.7)
1	0	0	118	43	64	0.0	0.2	5.9	ND	5
2	10	10	550	200	300	0.3	2.9	9.0	ND	4.5
3	160	150	1930	780	950	0.2	2.4	8.4	ND	NA
4	< 10	15	349	185	145	0.0	3.4	9.5	1,24	17
5	6	1	259	134	66	0.0	6.0	13.0	0.79	221
6	2	0	809	495	326	0.1	0.5	44.32	2.3	4
7	18	19	913	545	341	0.0	2.8	14.0	2.60	3
83	40	2	1335	546	761	0.1	1.9	9.7	ND	NA
9	ND	ND	ND	ND	ND	0.0	4.2	16.2	-	1
10	70	206	247	134	108	0.1	1.7	18.5	0.79	6
11	ND	ND	ND	ND	ND	0.0	0.9	9.5	2.11	10
12	ND	ND	ND	ND	ND	1.7	2.6	8.1	ND	1
134	28	13	1073	633	417	0.2	0.3	12.8	ND	0.5
14	8	11	676	323	341	0.1	1.2	11.9	1.14	NA

Abnormal values are given in bold

¹On Prednisolone 20 mg a day when these samples were taken

²Hypergammaglobulinemia on IVIG due to IgG2 deficiency

³The values are from the time at diagnosis of GATA2 deficiency 3 years ago

⁴The reference values for Patient 13 who was 12 years old at the time of HSCT are CD19 200–600, NK 70–1200, CD3 800–3500, cd4 400–1200, cd8 200–1200 (given in cells × 10⁶/L) and for IgG the normal reference value was 6.1–14.9 g/L

ND, not done; NA, not applicable