Characteristics of patients with GPA who developed a hematological malignancy.
| Cases | Total, n = 21 |
|---|---|
| Male, n (%) | 16 (75) |
| Female, n (%) | 5 (25) |
| Age at GPA onset, yrs, median (range) | 58 (25–69)1 |
| ANCA-positive ever, n (%) | 16/16 (100)2 |
| c-ANCA or PR3-ANCA | 12 |
| ANCA of unspecified type | 2 |
| P-ANCA or MPO-ANCA | 2 |
| Cytopenia > 1 yr prior to malignancy, n (%) | 16 (76) |
| Anemia, HB < 110 | 14 |
| Leukopenia, WBC < 3.0 | 10 |
| Thrombocytopenia, TPC < 130 | 8 |
| Cytopenia leading to discontinuation or decreased DMARD | 12 |
| BVAS at GPA diagnosis, median (range) | 21 (11–36)1 |
| VDI at time of hematological malignancy, median (range) | 5 (1–10)1 |
↵1 Range min–max.
↵2 C-ANCA/PR3-ANCA unknown in the 5 patients diagnosed before ANCA testing was available (1968–1982). GPA: granulomatosis with polyangiitis; ANCA: antineutrophil cytoplasmic antibody; c-ANCA: cytoplasmic ANCA; PR3-ANCA: proteinase-3 ANCA; P-ANCA: perinuclear ANCA; MPO-ANCA: myeloperoxidase ANCA; HB: hemoglobin; WBC: white blood cells; TPC: total platelet count; DMARD: disease-modifying antirheumatic drugs; BVAS: Birmingham Vasculitis Activity Score; VDI: Vasculitis Damage Index.