Serum protein levels and lymphocyte phenotype before and after eculizumab therapy
| Parameter | P5 | P6 | P8 | |||
|---|---|---|---|---|---|---|
| Pre-eculizumab | 45 wk after eculizumab | Pre-eculizumab | 8 wk after eculizumab | Pre-eculizumab | 29 wk after eculizumab | |
| Age at evaluation, years | 15 | 16 | 5 | 5 | 11 | 12 |
| Albumin, g/L | 19 | 45 | 12 | 41 | 23 | 45 |
| Complement proteins, mg/dl | ||||||
| C3 | 82 | 99 | 89 | 130 | 82 | 146 |
| C4 | 20 | 24 | 22 | 46 | 21 | 37 |
| Immunoglobulins, mg/dl | ||||||
| IgG | 178 | 992 | 122 | 981 | 201 | 1,210 |
| IgM | 30 | 228 | 32 | 122 | 26 | 203 |
| IgA | 74 | 80 | 51 | 183 | 41 | 223 |
| Lymphocyte phenotyping | ||||||
| Lymphocytes, cells/μl | 5,166 | 2,855 | 6,237 | 6,290 | 3,758 | 2,781 |
| T-B-NK enumeration, cells/μl | ||||||
| CD3+ T cells | 4,340 | 2,113 | 4,304 | 4,529 | 2,856 | 2,113 |
| CD4+ T cells | 2,170 | 1,256 | 2,245 | 2,013 | 1,654 | 1,279 |
| CD8+ T cells | 2,015 | 742 | 1,559 | 1,950 | 940 | 612 |
| CD4/CD8 ratio | 1.08 | 1.69 | 1.44 | 1.03 | 1.76 | 2.09 |
| CD19+ B cells | 207 | 343 | 686 | 692 | 752 | 473 |
| NK cells | 620 | 400 | 1,247 | 1,069 | 113 | 195 |
| Extended CD4+T cell phenotyping, % of CD4 (cells/μl) | ||||||
| CD4+CD45RA+ | 48 (1,042) | 35 (440) | 66 (1,482) | 62 (1,248) | 51 (844) | 47 (601) |
| Naïve CD4+ T cells (CD45RA+CCR7+) | 46 (998) | 35 (440) | 65 (1,459) | 62 (1,248) | 50 (827) | 46 (588) |
| Memory CD4+ T cells (CD45RO+) | 52 (1,128) | 65 (816) | 34 (763) | 38 (765) | 49 (810) | 53 (678) |
| Extended CD8+T cell phenotyping, % of CD8 (cells/μl) | ||||||
| CD8+CD45RA+ | 58 (1,169) | 55 (408) | 66 (1,029) | 60 (1,170) | 49 (461) | 52 (318) |
| Naïve CD8+ T cells (CD45RA+CCR7+) | 37 (746) | 32 (237) | 61 (951) | 54 (1,053) | 32 (301) | 32 (196) |
| Memory CD8+ T cells (CD8+CD45RO+) | 42 (846) | 45 (334) | 34 (530) | 40 (780) | 51 (479) | 48 (294) |
| Extended CD19+B cell phenotyping, % of CD19 (cells/μl) | ||||||
| Naïve B cells (CD27–IgD+) | 23.2 (48) | 80.7 (277) | 41.7 (286) | 61.4 (425) | 66.1 (497) | 68.8 (325) |
| Unswitched memory B cells (CD27+IgD+) | 25.5 (53) | 10.4 (36) | 19.9 (137) | 18.7 (129) | 10.6 (80) | 11.4 (54) |
| Switched memory B cells (CD27+IgD–) | 41.5 (86) | 6.0 (22) | 22.5 (154) | 11.4 (79) | 14.7 (111) | 12.9 (61) |
| Transitional B cells (CD24++CD38++) | 0.4 (1) | 7.6 (26) | 1.0 (7) | 5.9 (41) | 0.9 (7) | 5.8 (27) |
| CD21lo B cells (CD21lowCD38low) | 10.7 (22) | 2.1 (7) | 7.6 (52) | 5.3 (37) | 5.2 (39) | 3.1 (15) |
| Plasmablasts (CD24–CD38++) | 6.2 (13) | 1.8 (6) | 10.9 (75) | 5.9 (41) | 3.7 (28) | 3.2 (15) |
| Parameter | P5 | P6 | P8 | |||
|---|---|---|---|---|---|---|
| Pre-eculizumab | 45 wk after eculizumab | Pre-eculizumab | 8 wk after eculizumab | Pre-eculizumab | 29 wk after eculizumab | |
| Age at evaluation, years | 15 | 16 | 5 | 5 | 11 | 12 |
| Albumin, g/L | 19 | 45 | 12 | 41 | 23 | 45 |
| C3 | 82 | 99 | 89 | 130 | 82 | 146 |
| C4 | 20 | 24 | 22 | 46 | 21 | 37 |
| IgG | 178 | 992 | 122 | 981 | 201 | 1,210 |
| IgM | 30 | 228 | 32 | 122 | 26 | 203 |
| IgA | 74 | 80 | 51 | 183 | 41 | 223 |
| Lymphocytes, cells/μl | 5,166 | 2,855 | 6,237 | 6,290 | 3,758 | 2,781 |
| CD3+ T cells | 4,340 | 2,113 | 4,304 | 4,529 | 2,856 | 2,113 |
| CD4+ T cells | 2,170 | 1,256 | 2,245 | 2,013 | 1,654 | 1,279 |
| CD8+ T cells | 2,015 | 742 | 1,559 | 1,950 | 940 | 612 |
| CD4/CD8 ratio | 1.08 | 1.69 | 1.44 | 1.03 | 1.76 | 2.09 |
| CD19+ B cells | 207 | 343 | 686 | 692 | 752 | 473 |
| NK cells | 620 | 400 | 1,247 | 1,069 | 113 | 195 |
| CD4+CD45RA+ | 48 (1,042) | 35 (440) | 66 (1,482) | 62 (1,248) | 51 (844) | 47 (601) |
| Naïve CD4+ T cells (CD45RA+CCR7+) | 46 (998) | 35 (440) | 65 (1,459) | 62 (1,248) | 50 (827) | 46 (588) |
| Memory CD4+ T cells (CD45RO+) | 52 (1,128) | 65 (816) | 34 (763) | 38 (765) | 49 (810) | 53 (678) |
| CD8+CD45RA+ | 58 (1,169) | 55 (408) | 66 (1,029) | 60 (1,170) | 49 (461) | 52 (318) |
| Naïve CD8+ T cells (CD45RA+CCR7+) | 37 (746) | 32 (237) | 61 (951) | 54 (1,053) | 32 (301) | 32 (196) |
| Memory CD8+ T cells (CD8+CD45RO+) | 42 (846) | 45 (334) | 34 (530) | 40 (780) | 51 (479) | 48 (294) |
| Naïve B cells (CD27–IgD+) | 23.2 (48) | 80.7 (277) | 41.7 (286) | 61.4 (425) | 66.1 (497) | 68.8 (325) |
| Unswitched memory B cells (CD27+IgD+) | 25.5 (53) | 10.4 (36) | 19.9 (137) | 18.7 (129) | 10.6 (80) | 11.4 (54) |
| Switched memory B cells (CD27+IgD–) | 41.5 (86) | 6.0 (22) | 22.5 (154) | 11.4 (79) | 14.7 (111) | 12.9 (61) |
| Transitional B cells (CD24++CD38++) | 0.4 (1) | 7.6 (26) | 1.0 (7) | 5.9 (41) | 0.9 (7) | 5.8 (27) |
| CD21lo B cells (CD21lowCD38low) | 10.7 (22) | 2.1 (7) | 7.6 (52) | 5.3 (37) | 5.2 (39) | 3.1 (15) |
| Plasmablasts (CD24–CD38++) | 6.2 (13) | 1.8 (6) | 10.9 (75) | 5.9 (41) | 3.7 (28) | 3.2 (15) |