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Table 1 Patient characteristics

From: Angioedema due to acquired C1-inhibitor deficiency: spectrum and treatment with C1-inhibitor concentrate

  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)
  1. a. an additional 3 patients had autoantibodies to C1-INH and an associated disorder
  2. C1-INH = C1 inhibitor; pdC1-INH = plasma-derived C1-inhibitor concentrate; MGUS = monoclonal gammopathy of undetermined significance; SD = standard deviation