Characterizing T Cells in SCID Patients Presenting with Reactive or Residual T Lymphocytes

Table 1

Clinical and immunological findings in 6 patients diagnosed with SCID.

	Pt-1	Pt-2	Pt-3	Pt-4	Pt-5	Pt-6
Diagnosis, genetic defect	SCID-RAG2	SCID-𝛾𝑐	SCID-RAG2	SCID-RAG2	CID MHC-II	CID MHC-II
Age at diagnosis (months)	5/12	7/12	4/12	3/12	6/12	6/12
Maternal cells	100%	100%	3.5%	2%	0%	0%
Autoimmune features	Mild	Mild	Severe	Severe	No	No
Infections	+	+	+	+	+	+
Lymphocyte count/mm^3	5600	4500	1320	10686	4900	3416
Eosinophil/mm^3	1700	600	2960	4030	500	70
CD3/mm^3	4612	1500	488	2871	3552	1162
CD3^+CD4^+/mm^3	2855	360	244	2351	543	137
CD3^+CD8^+/mm^3	1757	1080	224	855	3305	1009
CD19^+/mm^3	1	3015	0	0	543	2186
CD3^−CD56^+/mm^3	504	0	500	3800	490	0
HLADR^+ (in total lymph)	47%	87%	30%	53%	0%	0%
IGM (IU/mL)	UD	UD	UD	110	UD	UD
IGG (IU/mL)	433	UD	UD	UD	253	UD
IGA (IU/mL)	79	UD	UD	UD	UD	UD
IGE (IU/mL)	UD	UD	UD	UD	UD	UD
PHA mitogenic response*	3.8%	2.4%	6%	6.5%	46.9%	94.8
aCD3 mitogenic response*	1.9%	ND	6.7%	31.5%	41.3%	32.8%
TRECs/0.5 mcg DNA	UD	UD	UD	UD	2769	4384

UD: undetectable, *percentage, CPM patient/CPM control, 𝛾_𝑐: common gamma chain, RAG: recombination activating gene.