Table 4.
Comparison of Puerta de Hierro PNH series with previous published series
| First author (yr) (reference) | Number of patients Period of study | Population site | Age of PNH diagnosis Sex | Survival & complications Spontaneous remission | Patients with aplastic anemia, % | Adverse prognostic factors | Number of deaths by cancer |
|---|---|---|---|---|---|---|---|
| Dacie (1972) & Hillmen (1995) 27,29 | 80 >30 yr | London (UK) Hammersmith Hospital | 42 (15–75) 33♂/47♀ | 60 dead Median survival 10 yr Hemoglobinuria 26% Thrombosis 39% 12 survivors with spontaneous remission | 29 | NR | 1 (lymphoma) |
| Kruatrachue (1978) 28 | 85 25 yr | Bangkok (Thailand) Siriraj Hospital | 30 (10–80) 52♂/23♀ | 7 dead Hemoglobinuria 32% Thrombosis 1% Abdominal pain <1% | 10 | NR | None |
| Socié (1996) 30 | 220 46 yr | France Multicenter | 33 (6–82) 100♂/120♀ | 71 dead Median survival 15 yr Thrombosis 6% Abdominal pain 13% | 30 | Age >55 Thrombosis MDS/AML Thrombocytopenia | 4 (3 MDS/AML) |
| Nishimura (2004) 31 | 385 36 yr (USA) 28 yr (Japan) | 176 USA (Duke University) 209 Japan (Multicenter) | 30 (4–80) 77♂/99♀ 45 (10–86) 118♂/91♀ | Median survival 19 and 32 yr Hemoglobinuria 41% >Thrombosis in USA 19% vs. 6% | 33 | Age >50 Leukopenia infection thrombosis (USA) Kidney failure (Japan) | 13 (9 MDS/AML) |
| Muñoz-Linares (2013) | 56 40 yr | Spain Puerta de Hierro Majadahonda Hospital | 32 (13–84) 36♂/20♀ | 12 dead Median survival 11 yr Hemoglobinuria 60% Thrombosis 35% Abdominal pain 48% 17 survivors with spontaneous remission | 67 | Thrombosis Esophageal spasms Abdominal pain | 5 (Pancreas, lung, hepatocarcinoma, and 2 lymphomas) |
AML, acute myeloid leukemia; MDS, myelodysplastic syndrome; NR, not reported; PNH, paroxysmal nocturnal hemoglobinuria.