Table 1 (abstract O-29) Clinical and biological characteristics of AAE and MGUS
From: 13th C1-inhibitor deficiency and angioedema workshop—2023
N | IgM | IgG | IgA | Total | |
|---|---|---|---|---|---|
MIg isotype, n (%) | 41 | 24 (58%) | 11 (27%) | 6 (15%) | 41 |
Mean mIg value, n (g/L) (SD) | 40 | 4 (4.2) | 6.7 (6.3) | 3.8 (1.7) | 4,8 (4,8) |
Sex ratio, F:M | 41 | 1/1 | 0,72/1 | 2/1 | 1/1 |
Mean age at 1st AE attack y (SD) | 41 | 64 (12) | 58 (11) | 68 (17) | 63 (13) |
Mean diagnostic delay, y (SD) | 41 | 3 (4) | 3 (5) | 1 (1) | 3 (4) |
Anti-C1INH Ab presence | 41 | 15 (63%) | 7 (64%) | 6 (100%) | 28 (68%) |
Anti-C1INH Ab isotype | 41 | 10 IgM 5 IgG | 7 IgG | 6 IgA | 6 IgA 10 IgM 12 IgG |
Low C1INH antigen | 40 | 22 (92%) | 8 (80%) | 5 (83%) | 35 (87%) |
Low C1q | 34 | 15 (94%) | 5 (55%) | 4 (100%) | 29 (85%) |
Low C4 | 41 | 24 (100%) | 9 (82%) | 6 (100%) | 39 (95%) |
Evolution into malignant hemopathy | 41 | 7 | 6 | 1 | 14 (34%) |
Mean delay between AAE diagnosis and evolution into malignant hemopathy (years) | 14 | 7 | 11 | 4 | 9 |