Table 1 Patient characteristics
All patients (N = 44) | pdC1-INH treated patients (N = 32) | |||
|---|---|---|---|---|
Age at first angioedema (years), mean (SD) | 56.2 | (14.8) | 56.0 | (14.7) |
< 40 (years), n (%) | 5 | (11.4) | 4 | (12.5) |
40 - < 50 (years), n (%) | 9 | (20.4) | 6 | (13.6) |
50 - < 60 (years), n (%) | 11 | (11.0) | 7 | (21.9) |
60 - < 70 (years), n (%) | 10 | (22.7) | 9 | (28.1) |
70 - < 80 (years), n (%) | 7 | (15.9) | 4 | (12.5) |
≥80 (years), n (%) | 2 | (4.5) | 2 | (6.3) |
Gender, n (%) | ||||
Male | 17 | (38.6) | 14 | (43.8) |
Female | 27 | (61.4) | 18 | (56.3) |
Associated disorders, n (%) | ||||
MGUS | 21 | (47.7) | 15 | (46.9) |
Non-Hodgkin lymphoma | 12 | (27.3) | 10 | (31.3) |
Splenic marginal cell lymphoma | 6 | (13.6) | 4 | (12.5) |
Plasmocytoma | 2 | (4.5) | 2 | (6.3) |
B-cell lymphoma | 1 | (2.3) | 1 | (3.1) |
Waldenström’s macroglobulinemia | 1 | (2.3) | 1 | (3.1) |
Centroblastic-centrocytic follicular lymphoma | 1 | (2.3) | 1 | (3.1) |
Diffuse anaplastic large cell B-cell lymphoma | 1 | (2.3) | 1 | (3.1) |
Anti-C1-INH autoantibodies with no other associated disorders a | 5 | (11.4) | 5 | (15.6) |
Other associated disorders | 2 | (4.5) | 0 | |
None | 4 | (9.1) | 2 | (6.3) |
Plasma complement, n (%) | ||||
C1-INH function | ||||
< normal range of 70–130% | 44 | (100) | 32 | (100) |
< 50% | 44 | (100) | 32 | (100) |
< 5% | 28 | (63.6) | 21 | (65.6) |
C1-INH protein | ||||
< normal range of 15.4–33.8 mg/dL | 44 | (100) | 32 | (100) |
< 12 mg/dL | 41 | (93.2) | 32 | (100) |
< 4.8 mg/dL | 27 | 61.4 | 20 | (62.5) |
C4 | ||||
< normal range of 16.4–31.3 mg/dL | 44 | (100) | 32 | (100) |
< 12 mg/dL | 43 | (97.7) | 32 | (100) |
< 4.8 mg/dL | 39 | (88.6) | 30 | (93.8) |
C1q | ||||
< normal range of 0.1–0.25 g/L | 39 | (88.6) | 29 | (90.6) |
< 0.05 g/L | 26 | (59.1) | 21 | (65.6) |