Immunological parameters of the monoallelic PSMB10 G209R patient over time
| . | 1 wk . | 13 mo . | 25 mo . | 38 mo . | 51 mo . | 66 mo . |
|---|---|---|---|---|---|---|
| White blood cells, cells/µl | 5,818 (7,540–14,910) | 8,324 (6,510–13,940) | 9,333 (5,420–11,870) | 10,228 (5,420–11,870) | 8,300 (5,420–11,870) | 7,277 (5,420–11,870) |
| Lymphocytes, cells/µl | 1,923 (3,500–13,100) | 3,578 (2,600–10,400) | 3,939 (2,700–11,900) | 2,139 (1,700–6,900) | 3,184 (1,700–6,900) | 2,453 (1,100–5,900) |
| Neutrophils, cells/µl | 1,086 (1,960–4,710) | 2,965 (1,400–4,550) | 3,555 (1,790–5,640) | 6,995 (1,790–5,640) | 3,121 (1,790–5,640) | 3,738 (1,790–5,640) |
| Eosinophils, cells/µl | 254 (20–720) | 1,034 (60–430) | 526 (70–500) | 226 (70–500) | 817 (70–500) | 263 (70–500) |
| Monocytes, cells/µl | 596 (570–1,900) | 429 (490–1,170) | 399 (440–950) | 574 (440–950) | 460 (440–950) | 391 (440–950) |
| Total T cells (CD3), cells/µl | 208 (2,300–7,000) | 919 (1,600–6,700) | 1,367 (1,400–8,000) | 903 (900–4,500) | 1,676 (900–4,500) | 1,261 (700–4,200) |
| T helper cells (CD4), cells/µl | 134 (1,700–5,300) | 603 (1,000–4,600) | 957 (900–5,500) | 612 (500–2,400) | 984 (500–2,400) | 721 (300–2,000) |
| Naïve CD4 T cells (CD45RA), cells/µl | 38 | 253 | 428 | 262 | 401 | 275 |
| Naïve CD4 T cells (CD45RA), % | 28.3 (81.0–91.5) | 41.9 (75.8–88.1) | 44.7 (61.8–85.0) | 42.8 (61.8–85.0) | 40.7 (61.8–85.0) | 38.2 (61.8–85.0) |
| Memory CD4 T cells (CD45RO), cells/µl | 92 | 281 | 395 | 306 | 488 | 465 |
| Memory CD4 T cells (CD45RO), % | 68.6 (5.1–11.4) | 46.6 (10.0–15.6) | 41.3 (14.8–37.2) | 50.0 (14.8–37.2) | 49.6 (14.8–37.2) | 64.5 (14.8–37.2) |
| Cytotoxic T cells (CD8), cells/µl | 18 (400–1,700) | 149 (400–2,100) | 262 (400–2,300) | 151 (300–1,600) | 427 (300–1,600) | 435 (300–1,800) |
| Naïve CD8 T cells (CD45RA), cells/µl | 14 | 75 | 124 | 79 | 237 | 214 |
| Memory CD8 T cells (CD45RO), cells/µl | 4 | 68 | 110 | 82 | 223 | 216 |
| CD4/CD8 ratio | 7:1 | 4:1 | 3,6:1 | 4:1 | 2,3:1 | 1,7:1 |
| TCRab T cells, cells/µl | 138 | 719 | 1,235 | 797 | 1,394 | 1,140 |
| TCRgd T cells, cells/μl | 5 | 115 | 121 | 73 | 192 | 148 |
| TRECS/106 T cells | | | | | 1.988 | |
| NK cells (CD3−CD56+CD16+), cells/µl | 254 (200–1,400) | 865 (200–1,200) | 632 (100–1,400) | 285 (100–1,000) | 267 (100–1,000) | 492 (90–900) |
| Total B cells (CD20), cells/µl | 423 (600–1,900) | 1,822 (773–1,990) | 1,832 (529–1,930) | 631 (529–1,930) | 1,141 (323–1,000) | 512 (323–1,000) |
| Naïve B cells (IgD+CD27−), cells/µl | 399 | 1,691 (574–2,215) | 1,602 (419–1,748) | 504 (419–1,748) | 829 (245–799) | 343 (245–799) |
| Intermediate B cells (IgD+CD27+), cells/µl | 7 | 67 (43–118) | 78 (39–208) | 45 (39–208) | 155 (29–113) | 51 (29–113) |
| Memory B cells (IgD−CD27+), cells/μl | 1 | 34 (13–54) | 99 (20–115) | 62 (20–115) | 126 (18–94) | 96 (18–94) |
| IgM (mg/dl) | 40 (3–32) | 66 (14–82) | 165 (36–144) | 134 (36–144) | 183 (36–144) | 189 (36–144) |
| IgG (mg/dl) | 986 (390–1,050) | 610 (470–1,230) | 776 (540–1,340) | 818 (540–1,340) | 904 (590–1,430) | 888 (590–1,430) |
| IgA (mg/dl) | <5 (<14) | 28 (<14) | 54 (<80) | 81 (11–142) | 92 (11–142) | 106 (11–142) |
| IgE (U/ml) | 22 (<2) | 26 (<60) | 12 (<60) | 14 (<60) | 11 (<60) | n.d. |
| Vaccine serology inactivated | n.d. | T+D+HepB+ | T+D+HepB+ | T+D+HepB+ | T+D+HepB+ | T+D+HepB+ |
| Live | | | M+Mu+R+V+ | M+Mu+R+V+ | M+Mu+R+V+ | M+Mu+R+V+ |
| . | 1 wk . | 13 mo . | 25 mo . | 38 mo . | 51 mo . | 66 mo . |
|---|---|---|---|---|---|---|
| White blood cells, cells/µl | 5,818 (7,540–14,910) | 8,324 (6,510–13,940) | 9,333 (5,420–11,870) | 10,228 (5,420–11,870) | 8,300 (5,420–11,870) | 7,277 (5,420–11,870) |
| Lymphocytes, cells/µl | 1,923 (3,500–13,100) | 3,578 (2,600–10,400) | 3,939 (2,700–11,900) | 2,139 (1,700–6,900) | 3,184 (1,700–6,900) | 2,453 (1,100–5,900) |
| Neutrophils, cells/µl | 1,086 (1,960–4,710) | 2,965 (1,400–4,550) | 3,555 (1,790–5,640) | 6,995 (1,790–5,640) | 3,121 (1,790–5,640) | 3,738 (1,790–5,640) |
| Eosinophils, cells/µl | 254 (20–720) | 1,034 (60–430) | 526 (70–500) | 226 (70–500) | 817 (70–500) | 263 (70–500) |
| Monocytes, cells/µl | 596 (570–1,900) | 429 (490–1,170) | 399 (440–950) | 574 (440–950) | 460 (440–950) | 391 (440–950) |
| Total T cells (CD3), cells/µl | 208 (2,300–7,000) | 919 (1,600–6,700) | 1,367 (1,400–8,000) | 903 (900–4,500) | 1,676 (900–4,500) | 1,261 (700–4,200) |
| T helper cells (CD4), cells/µl | 134 (1,700–5,300) | 603 (1,000–4,600) | 957 (900–5,500) | 612 (500–2,400) | 984 (500–2,400) | 721 (300–2,000) |
| Naïve CD4 T cells (CD45RA), cells/µl | 38 | 253 | 428 | 262 | 401 | 275 |
| Naïve CD4 T cells (CD45RA), % | 28.3 (81.0–91.5) | 41.9 (75.8–88.1) | 44.7 (61.8–85.0) | 42.8 (61.8–85.0) | 40.7 (61.8–85.0) | 38.2 (61.8–85.0) |
| Memory CD4 T cells (CD45RO), cells/µl | 92 | 281 | 395 | 306 | 488 | 465 |
| Memory CD4 T cells (CD45RO), % | 68.6 (5.1–11.4) | 46.6 (10.0–15.6) | 41.3 (14.8–37.2) | 50.0 (14.8–37.2) | 49.6 (14.8–37.2) | 64.5 (14.8–37.2) |
| Cytotoxic T cells (CD8), cells/µl | 18 (400–1,700) | 149 (400–2,100) | 262 (400–2,300) | 151 (300–1,600) | 427 (300–1,600) | 435 (300–1,800) |
| Naïve CD8 T cells (CD45RA), cells/µl | 14 | 75 | 124 | 79 | 237 | 214 |
| Memory CD8 T cells (CD45RO), cells/µl | 4 | 68 | 110 | 82 | 223 | 216 |
| CD4/CD8 ratio | 7:1 | 4:1 | 3,6:1 | 4:1 | 2,3:1 | 1,7:1 |
| TCRab T cells, cells/µl | 138 | 719 | 1,235 | 797 | 1,394 | 1,140 |
| TCRgd T cells, cells/μl | 5 | 115 | 121 | 73 | 192 | 148 |
| TRECS/106 T cells | | | | | 1.988 | |
| NK cells (CD3−CD56+CD16+), cells/µl | 254 (200–1,400) | 865 (200–1,200) | 632 (100–1,400) | 285 (100–1,000) | 267 (100–1,000) | 492 (90–900) |
| Total B cells (CD20), cells/µl | 423 (600–1,900) | 1,822 (773–1,990) | 1,832 (529–1,930) | 631 (529–1,930) | 1,141 (323–1,000) | 512 (323–1,000) |
| Naïve B cells (IgD+CD27−), cells/µl | 399 | 1,691 (574–2,215) | 1,602 (419–1,748) | 504 (419–1,748) | 829 (245–799) | 343 (245–799) |
| Intermediate B cells (IgD+CD27+), cells/µl | 7 | 67 (43–118) | 78 (39–208) | 45 (39–208) | 155 (29–113) | 51 (29–113) |
| Memory B cells (IgD−CD27+), cells/μl | 1 | 34 (13–54) | 99 (20–115) | 62 (20–115) | 126 (18–94) | 96 (18–94) |
| IgM (mg/dl) | 40 (3–32) | 66 (14–82) | 165 (36–144) | 134 (36–144) | 183 (36–144) | 189 (36–144) |
| IgG (mg/dl) | 986 (390–1,050) | 610 (470–1,230) | 776 (540–1,340) | 818 (540–1,340) | 904 (590–1,430) | 888 (590–1,430) |
| IgA (mg/dl) | <5 (<14) | 28 (<14) | 54 (<80) | 81 (11–142) | 92 (11–142) | 106 (11–142) |
| IgE (U/ml) | 22 (<2) | 26 (<60) | 12 (<60) | 14 (<60) | 11 (<60) | n.d. |
| Vaccine serology inactivated | n.d. | T+D+HepB+ | T+D+HepB+ | T+D+HepB+ | T+D+HepB+ | T+D+HepB+ |
| Live | | | M+Mu+R+V+ | M+Mu+R+V+ | M+Mu+R+V+ | M+Mu+R+V+ |
Reference intervals according to (16) white blood cells, neutrophils, eosinophils, monocytes (17), and lymphocytes; total T, CD4 helper, cytotoxic CD8, and NK and B cells (18); naïve and memory CD4 percentages; and (19) B cell subpopulations and (20) TREC levels. Reduced cell counts are indicated in bold. Cell counts without reference ranges are indicated in italics. Ig reference intervals according to the local standard (University Hospital Duesseldorf). Abbreviations: T: tetanus; D: diphtheria; Hep B: hepatitis B; M: measles; Mu: mumps; R: rubella; V: varicella.