Demographic |
Male | 190 (96) | 197 |
Age at onset, yr, median (range) | 71 (43–93) | 197 |
Deceased at last follow-up | 52 (28) | 189 |
Clinical§ |
Skin lesions (e.g., neutrophilic dermatosis, vasculitis) | 161 (82) | 197 |
Fever | 142 (72) | 197 |
Pulmonary involvement (e.g., infiltrates, effusions) | 101 (51) | 197 |
Chondritis (e.g., ear, nose) | 90 (49) | 197 |
Constitutional (e.g., weight loss, fatigue) | 83 (54) | 154 |
Venous thromboembolism | 68 (37) | 185 |
Arthritis and arthralgias | 52 (30) | 172 |
Pathologic |
Myelodysplastic syndrome | 97 (49) | 197 |
Monoclonal gammopathy or multiple myeloma | 22 (11) | 197 |
Complete blood count parameters |
Hemoglobin, g/L, median (range) | 101 (68–139) | 144 |
Mean corpuscular volume, fL, median (range) | 101 (82–111) | 128 |
White blood cell count, × 109/L, median (range) | 4.5 (1.6–9.7) | 128 |
Absolute neutrophil count, × 109/L, median (range) | 2.6 (1.1–12.1) | 144 |
Platelet count, × 109/L, median (range) | 204 (36–428) | 144 |
C-reactive protein, mg/L, median (range) | 61 (19–407) | 163 |
Mutation type |
p.Met41Thr | 93 (47) | 197 |
p.Met41Val | 55 (28) | 197 |
p.Met41Leu | 37 (19) | 197 |
Splice site | 10 (5) | 197 |
p.Ser56Phe | 1 (1) | 197 |