Table 1 Epidemiological, clinical, pulmonary function, hematology parameters, outcome and genetic background of 5 Greek patients with IPF and MDS
From: Myelodysplastic syndromes and idiopathic pulmonary fibrosis: a dangerous liaison
Parameters | #1 | #2 | #3 | #4 | #5 |
|---|---|---|---|---|---|
Gender | F | M | M | M | M |
Age at IPF diagnosis (years) | 74 | 82 | 74 | 78 | 84 |
Age at MDS diagnosis (years) | 78 | 81 | 80 | 79 | 83 |
Age at death (years) | – | 82 | 81 | 79 | 84 |
Duration of treatment with azacytidine (months) | 0 | 14 | 9 | 1 | 0 |
Status | Alive | Dead | Dead | Dead | Dead |
Time to death from diagnosis of IPF (months) | – | 3 | 77 | 24 | 1 |
HRCT pattern | UIP | UIP | Probable UIP | Probable UIP | Probable UIP |
Lung histology | – | – | UIP | – | UIP |
Serology for CTD | negative | negative | negative | negative | negative |
FVC % pred | 88.6 | – | 80.8 | 70.5 | 68 |
DLCO % pred | 59.2 | – | 58.3 | 77 | 65 |
GAP stage | I | III | I | II | II |
WHO 2008 classification | RARS | RAEB II | RAEB I | RAEB I | RA |
IPSS score | Low | Intermediate 2 | Very high | Intermediate 1 | Low |
Treatment | – | Azacytidine | Azacytidine | Azacytidine | – |
Cycles azacytidine | – | 6 | 2 | 6 | – |
PO2/FiO2 | 357 | 118 | 137.5 | 142 | 73 |
Ht % | 33.9 | 26.2 | 29.4 | 37.9 | 34.3 |
MCV (fL) | 109.4 | 78.2 | 116.5 | 105.6 | 90.3 |
Neutrophils (G/L) | 3210 | 600 | 540 | 1210 | 6700 |
PLT (G/L) | 300 | 91 | 171 | 137 | 365 |
CRP (mg/L) | < 3 | 49.5 | 100 | 94.8 | 100 |
Family history | Yesa | No | No | No | No |
TRG mutation | No | No | No | No | No |
Telomere length (Kb) | 10.9 | NA | NA | NA | 9.1 |