Table 1 Patients’ demographics and clinical characteristics at enrollment into the International PNH Registry, Taiwan versus the rest of the world (ROW)
From: Characteristics of Taiwanese patients of PNH in the international PNH registry
Parameter | Taiwan Patients (n = 63) | ROW (n = 3857) | p-value |
|---|---|---|---|
Age, years, median (range) | 46.0 (9, 84) | 43.0 (0, 104) | 0.331 |
Females, n (%) | 31 (49.2) | 2072 (53.7) | 0.525 |
Age at disease start, years, median (range) | 37.5 (8, 84) (n = 62) | 34.0 (0, 90) (n = 3773) | 0.483 |
Disease duration, years, median (range) | 4.5 (0.1, 34.8) (n = 62) | 3.0 (0.0, 100.4) (n = 3773) | 0.890 |
GPI-Deficient Granulocytes, n (%) | (n = 11) | (n = 1619) | 0.980 |
< 10 % | 3 (27.3) | 482 (29.8) | |
> =10 % to < 50 % | 1 (9.1) | 317 (19.6) | |
> = 50 % | 7 (63.6) | 820 (50.6) | |
LDH Ratio (xULN), median (Q1, Q3) | 4.9 (1.9, 9.1) (n = 25) | 2.0 (1.0, 5.2) (n = 2398) | 0.005* |
> = 1.5, n (%) | 20 (80.0) | 1406 (58.6) | 0.039* |
Hemoglobin (g/L), median (Q1, Q3) | 80.0 (68.0, 105.0) (n = 51) | 99.0 (84.0, 118.0) (n = 2996) | <0.001* |
Platelets (x109/L), median (Q1, Q3) | 112.0 (51.0, 186.0) (n = 51) | 114.0 (51.0, 176.0) (n = 2990) | 0.680 |
Serum Creatinine (umol/L), median (Q1, Q3) | 66.3 (53.0, 97.2) (n = 36) | 73.0 (61.9, 91.0) (n = 2746) | 0.36 |
Proteinuria, n (%) | 0.056 | ||
Trace | 2 (12.5) | 93 (10.0) | |
1+ to 4+ | 6 (37.5) | 158 (16.9) | |
eGFR (mL/min), median (Q1, Q3) | 95.8 (70.1, 116.5) (n = 36) | 95.6 (73.9, 114.1) (n = 2638) | 0.994 |
< 30, n (%) | 3 (8.3) | 62 (2.4) | 0.143 |
30– < 60 | 3 (8.3) | 329 (12.5) | |
60– < 90 | 8 (22.2) | 726 (27.5) | |
> =90 | 22 (61.1) | 1521 (57.7) | |
Neutrophils (x 109/L), median (Q1, Q3) | 2.0 (1.0, 2.9) (n = 27) | 1.9 (1.1, 2.9) (n = 2674) | 0.696 |
History of Abdominal Pain, n (%) | 17 (31.5) (n = 54) | 943 (33.5) (n = 2817) | 0.884 |
History of Dysphagia, n (%) | 7 (13.0) (n = 54) | 434 (15.4) (n = 2812) | 0.848 |
History of Shortness of Breath, n (%) | 32 (59.3) (n = 54) | 1180 (41.9) (n = 2819) | 0.012* |
History of Erectile Dysfunction, n (%) | 17 (60.7) (n = 28) | 271 (23.0) (n = 1180) | <0.001* |
History of Fatigue, n (%) | 44 (81.5) (n = 54) | 2214 (78.5) (n = 2820) | 0.738 |
History of Hemoglobinuria, n (%) | 37 (68.5) (n = 54) | 1288 (45.7) (n = 2816) | 0.001* |
History of Bone Marrow Disorder, n (%) | 36 (58.1) (n = 62) | 2265 (60.2) (n = 3762) | 0.794 |
History of TE, n (%) | 4 (6.7) (n = 60) | 502 (13.5) (n = 3710) | 0.178 |
History of any RBC Transfusion, n (%) | 40 (67.8) (n = 59) | 1816 (57.0) (n = 3185) | 0.274 |
History of Impaired Renal Function, n (%) | 7 (12.1) (n = 58) | 456 (12.6) (n = 3609) | >0.999 |
History of Pulmonary Hypertension, n (%) | 0 (0.0) (n = 58) | 68 (1.9) (n = 3670) | 0.627 |
Ever Treated with Eculizumab, n (%) | 25 (39.7) | 1666 (43.2) | 0.610 |