Table 2.

Lymphocyte subsets in ALPS Ia families, probands with ALPS II/III, and HCs

Lymphocyte subsetProband Ia
(n = 31)
M+/A+
(n = 28)
M+/A
(n = 42)
M/A
(n = 65)
Proband II/III
(n = 11)
HC
(n = 40)
T cells       
 CD3+ 2239* [70.91768↑ [74.1] 1159 [76.7] 1487↑ [76.1] 2138↑ [71.5] 1105 [72.2] 
 CD4+ 824 [27.5] 712 [34.3] 654 [43.2] 841 [44.6] 800 [27.2] 723 [47.1] 
 CD8+ 1067↑ [26.1] 708↑ [29.8] 538↑ [34.1] 621↑ [32.0] 831↑ [30.7] 488 [29.8] 
 α/β+-DNT 198↑ [7.3] 117↑ [5.9] 17↑ [0.8] 13↑ [0.5] 66↑ [3.5] [0.5] 
 γ/δ+-DNT 93↑ [2.6] 47↑ [1.5] 33↑ [1.9] 32↑ [1.7] 53↑ [1.9] 18 [1.1] 
 CD3+/HLA-DR+ 705↑ [21.7] 522↑ [20.2] 201 [11.6] 187 [9.3] 374↑ [15.2] 146 [8.8] 
 CD3+/CD25+ 270↓ [7.4] 378 [17.3] 411 [24.8] 447 [25.4] 211↓ [9.2] 425 [27.6] 
 CD8+/CD57+ 444↑ [11.3] 291↑ [11.9] 206 [12.4] 246 [12.0] 297↑ [11.3] 206 [12.5] 
B cells 
 CD20+ 656↑ [20.8] 295↑ [13.3] 148 [9.2] 235↑ [11.6] 436↑ [14.5] 148 [9.8] 
 CD5+/CD20+ 526↑ [13.0] 190↑ [6.5] 70 [4.3] 114↑ [5.6] 339↑ [10.6] 56 [3.3] 
NK cells 
 CD3CD16+ and/or 56+ 225 [7.6] 224 [10.1] 209 [11.2] 219 [9.7] 229 [10.5] 212 [15]  
Lymphocyte subsetProband Ia
(n = 31)
M+/A+
(n = 28)
M+/A
(n = 42)
M/A
(n = 65)
Proband II/III
(n = 11)
HC
(n = 40)
T cells       
 CD3+ 2239* [70.91768↑ [74.1] 1159 [76.7] 1487↑ [76.1] 2138↑ [71.5] 1105 [72.2] 
 CD4+ 824 [27.5] 712 [34.3] 654 [43.2] 841 [44.6] 800 [27.2] 723 [47.1] 
 CD8+ 1067↑ [26.1] 708↑ [29.8] 538↑ [34.1] 621↑ [32.0] 831↑ [30.7] 488 [29.8] 
 α/β+-DNT 198↑ [7.3] 117↑ [5.9] 17↑ [0.8] 13↑ [0.5] 66↑ [3.5] [0.5] 
 γ/δ+-DNT 93↑ [2.6] 47↑ [1.5] 33↑ [1.9] 32↑ [1.7] 53↑ [1.9] 18 [1.1] 
 CD3+/HLA-DR+ 705↑ [21.7] 522↑ [20.2] 201 [11.6] 187 [9.3] 374↑ [15.2] 146 [8.8] 
 CD3+/CD25+ 270↓ [7.4] 378 [17.3] 411 [24.8] 447 [25.4] 211↓ [9.2] 425 [27.6] 
 CD8+/CD57+ 444↑ [11.3] 291↑ [11.9] 206 [12.4] 246 [12.0] 297↑ [11.3] 206 [12.5] 
B cells 
 CD20+ 656↑ [20.8] 295↑ [13.3] 148 [9.2] 235↑ [11.6] 436↑ [14.5] 148 [9.8] 
 CD5+/CD20+ 526↑ [13.0] 190↑ [6.5] 70 [4.3] 114↑ [5.6] 339↑ [10.6] 56 [3.3] 
NK cells 
 CD3CD16+ and/or 56+ 225 [7.6] 224 [10.1] 209 [11.2] 219 [9.7] 229 [10.5] 212 [15]  

Bold indicates statistically significant change compared to the HC group.

↑Absolute number higher than HC group.

↓Absolute number lower than HC group.

*

Median number of cells/μL.

Median percentage of lymphocytes.

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