Table 2. Phenotypic characterization of... | American Society of Hematology

Table 2.

Phenotypic characterization of the lymphocyte compartment and somatic hypermutations in ICOS deficiency

	Age at testing, y	Lymphocyte count, × 10⁹/L	CD3, %Ly	CD4, %Ly	CD8, %Ly	CD19, %Ly	CD19/μL	CD27^-IgD⁺, %Bc	CD27⁺IgD⁺, %Bc	CD27⁺IgD^-, %Bc	SHM, %bp
Family A
Patient 1	41	1.266	95.6*	59.2*	31.9	0.6*	7*	89.6*	5.9*	0.8*	ND
Patient 2	32	1.047	88.5*	39.9	47.2*	0.6*	9*	88.5*	7.8*	1.6*	3.8
Family B
Patient 3	35	1.171	77.6	50.5	22.8	2.8*	34*	94.9*	4.1*	0.2*	—†
Patient 4	25	0.353*	91.9*	44.8	33.6	3.2*	5*	92.9*	5.1*	0.3*	ND
Family C
Patient 5	46	0.871*	72.2	55.1	16.6	14.6	127	90.4*	9	0.2*	2.2*
Patient 6	42	1.722	91.9*	23.1	64.0*	2.01*	33*	90.2*	11.3	1.3*	ND
Family D
Patient 7	18	3.031	88.5*	40.8	47.5*	6.1	184	96.8*	2.7*	0*	—†
Patient 8	5	3.584	76.1	39.0	34.5	19.1	686	95.2*	3.2*	1.3*	2.3*
Patient 9	5	4.153	70.4	32.8	34.8	21.2	882	97.7*	2*	0*	—†
Mean ± SD	—	1.910 ± 1.262	83.6 ± 9.0	42.8 ± 10.5	37.0 ± 13.4	7.8 ± 7.7	219 ± 311	92.9 ± 3.2	5.7 ± 3.0	0.6 ± 0.6	2.8 ± 0.7
Normal range	—	1.0-2.8	55-83	28-57	10-39	6-19	100-500	38-80	7.9-36	6.5-33	2.5-6.3

	Age at testing, y	Lymphocyte count, × 10⁹/L	CD3, %Ly	CD4, %Ly	CD8, %Ly	CD19, %Ly	CD19/μL	CD27^-IgD⁺, %Bc	CD27⁺IgD⁺, %Bc	CD27⁺IgD^-, %Bc	SHM, %bp
Family A
Patient 1	41	1.266	95.6*	59.2*	31.9	0.6*	7*	89.6*	5.9*	0.8*	ND
Patient 2	32	1.047	88.5*	39.9	47.2*	0.6*	9*	88.5*	7.8*	1.6*	3.8
Family B
Patient 3	35	1.171	77.6	50.5	22.8	2.8*	34*	94.9*	4.1*	0.2*	—†
Patient 4	25	0.353*	91.9*	44.8	33.6	3.2*	5*	92.9*	5.1*	0.3*	ND
Family C
Patient 5	46	0.871*	72.2	55.1	16.6	14.6	127	90.4*	9	0.2*	2.2*
Patient 6	42	1.722	91.9*	23.1	64.0*	2.01*	33*	90.2*	11.3	1.3*	ND
Family D
Patient 7	18	3.031	88.5*	40.8	47.5*	6.1	184	96.8*	2.7*	0*	—†
Patient 8	5	3.584	76.1	39.0	34.5	19.1	686	95.2*	3.2*	1.3*	2.3*
Patient 9	5	4.153	70.4	32.8	34.8	21.2	882	97.7*	2*	0*	—†
Mean ± SD	—	1.910 ± 1.262	83.6 ± 9.0	42.8 ± 10.5	37.0 ± 13.4	7.8 ± 7.7	219 ± 311	92.9 ± 3.2	5.7 ± 3.0	0.6 ± 0.6	2.8 ± 0.7
Normal range	—	1.0-2.8	55-83	28-57	10-39	6-19	100-500	38-80	7.9-36	6.5-33	2.5-6.3

Lymphocyte subpopulations were analyzed by flow cytometry with the following markers: CD3 for total T cells and CD4 and CD8 for the respective subpopulations, CD19 for B cells with naive (CD27^-IgD⁺), IgM memory (CD27⁺IgD⁺), and switched memory (CD27⁺IgD^-) subpopulations. Normal ranges are determined for adult population.

%Ly indicates percentage of lymphocytes; %Bc, percentage of B cells; SHM, somatic hypermutation; %bp, percentage of mutated base pairs; ND, not determined; —, not applicable.

Pathologic values.

†

The percentage of CD19⁺CD27⁺ cells was too low for analysis.

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