Immunologic Features of Nine Patients With ALPS
| . | Patient No. . | Normal Range . | ||||||||
|---|---|---|---|---|---|---|---|---|---|---|
| . | 1 . | 2 . | 3 . | 4 . | 5 . | 6 . | 14 . | 17 . | 20 . | . |
| Lymphocyte phenotype (cells/μL [% of lymphocytes]) | ||||||||||
| PBL | 13,344 | 9,176 | 13,370 | 2,074 | 2,470 | 12,328 | 5,152 | 3,286 | 975 | 473-4,800 |
| T cells | ||||||||||
| CD3+ | 9,434 (71) | 6,830 (65) | 9,586 (72) | 1,429 (69) | 1,756 (71) | 8,297 (67) | 2,880 (56) | 2,160 (59) | 857 (88) | 832-2,028 (61-84) |
| CD4+ | 2,936 (22) | 1,471 (14) | 3,610 (27) | 519 (25) | 553 (22) | 3,464 (28) | 927 (18) | 961 (26) | 390 (40) | 480-1,339 (33-59) |
| CD8+ | 2,509 (19) | 1,923 (18) | 4,158 (31) | 697 (34) | 778 (31) | 3,292 (27) | 876 (17) | 745 (20) | 343 (35) | 351-911 (18-47) |
| CD3+DR+ | 5,885 (44) | 3,499 (33) | ND | 243 (12) | 447 (18) | 4,056 (33) | 1,159 (23) | 1,104 (30) | 239 (25) | <15* |
| CD3+CD4−8− TCR α/β+ | 3,746 (28) | 2,144 (20) | 2,005 (15) | 79 (4) | 452 (18) | 1,566 (13) | 1,031 (20) | 395 (11) | 146 (15) | <1%* |
| CD3+CD4−8− TCR γ/δ+ | 227 (2) | 725 (7) | 13 (0.1) | 114 (6) | 41 (7) | 999 (8) | 155 (3) | 29 (1) | 25 (3) | <10%* |
| NK cells | ||||||||||
| CD16+/56+ | 400 (3) | 494 (5) | 521 (4) | 97 (5) | 101 (4) | 2,120 (17) | 402 (8) | 303 (8) | 47 (5) | 120-490 (6.5-30) |
| B cells | ||||||||||
| CD19+ | 3,216 (24) | 2,911 (28) | 3,155 (23) | 539 (27) | 652 (26) | 1,701 (14) | 2,524 (49) | 1,155 (32) | 107 (11) | 94-378 (6-16) |
| Lymphocyte phenotype (percent of CD3+ cells) | ||||||||||
| Lymphoid tissue† | ||||||||||
| CD4− CD8− | ND | 50 | 23 | 25 | 50 | 46 | 62 | ND | ND | |
| Serum Igs (mg/dL) | ||||||||||
| IgM | 77 | 400 | 40 | 41 | 330 | 240 | 126 | 132 | 30 | 37-200 |
| IgG | 1,480 | 4,020 | 1,700 | 2,040 | 1,700 | 2,300 | 600 | 1,650 | 1,070 | 523-1,482 |
| IgA | 700 | 1,670 | 310 | 1,800 | <10 | 500 | 870 | 360 | 159 | 51-375 |
| . | Patient No. . | Normal Range . | ||||||||
|---|---|---|---|---|---|---|---|---|---|---|
| . | 1 . | 2 . | 3 . | 4 . | 5 . | 6 . | 14 . | 17 . | 20 . | . |
| Lymphocyte phenotype (cells/μL [% of lymphocytes]) | ||||||||||
| PBL | 13,344 | 9,176 | 13,370 | 2,074 | 2,470 | 12,328 | 5,152 | 3,286 | 975 | 473-4,800 |
| T cells | ||||||||||
| CD3+ | 9,434 (71) | 6,830 (65) | 9,586 (72) | 1,429 (69) | 1,756 (71) | 8,297 (67) | 2,880 (56) | 2,160 (59) | 857 (88) | 832-2,028 (61-84) |
| CD4+ | 2,936 (22) | 1,471 (14) | 3,610 (27) | 519 (25) | 553 (22) | 3,464 (28) | 927 (18) | 961 (26) | 390 (40) | 480-1,339 (33-59) |
| CD8+ | 2,509 (19) | 1,923 (18) | 4,158 (31) | 697 (34) | 778 (31) | 3,292 (27) | 876 (17) | 745 (20) | 343 (35) | 351-911 (18-47) |
| CD3+DR+ | 5,885 (44) | 3,499 (33) | ND | 243 (12) | 447 (18) | 4,056 (33) | 1,159 (23) | 1,104 (30) | 239 (25) | <15* |
| CD3+CD4−8− TCR α/β+ | 3,746 (28) | 2,144 (20) | 2,005 (15) | 79 (4) | 452 (18) | 1,566 (13) | 1,031 (20) | 395 (11) | 146 (15) | <1%* |
| CD3+CD4−8− TCR γ/δ+ | 227 (2) | 725 (7) | 13 (0.1) | 114 (6) | 41 (7) | 999 (8) | 155 (3) | 29 (1) | 25 (3) | <10%* |
| NK cells | ||||||||||
| CD16+/56+ | 400 (3) | 494 (5) | 521 (4) | 97 (5) | 101 (4) | 2,120 (17) | 402 (8) | 303 (8) | 47 (5) | 120-490 (6.5-30) |
| B cells | ||||||||||
| CD19+ | 3,216 (24) | 2,911 (28) | 3,155 (23) | 539 (27) | 652 (26) | 1,701 (14) | 2,524 (49) | 1,155 (32) | 107 (11) | 94-378 (6-16) |
| Lymphocyte phenotype (percent of CD3+ cells) | ||||||||||
| Lymphoid tissue† | ||||||||||
| CD4− CD8− | ND | 50 | 23 | 25 | 50 | 46 | 62 | ND | ND | |
| Serum Igs (mg/dL) | ||||||||||
| IgM | 77 | 400 | 40 | 41 | 330 | 240 | 126 | 132 | 30 | 37-200 |
| IgG | 1,480 | 4,020 | 1,700 | 2,040 | 1,700 | 2,300 | 600 | 1,650 | 1,070 | 523-1,482 |
| IgA | 700 | 1,670 | 310 | 1,800 | <10 | 500 | 870 | 360 | 159 | 51-375 |