Table 1.

Characteristics of patients with GPA who developed a hematological malignancy.

CasesTotal, 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-ANCA12
  ANCA of unspecified type2
  P-ANCA or MPO-ANCA2
Cytopenia > 1 yr prior to malignancy, n (%)16 (76)
  Anemia, HB < 11014
  Leukopenia, WBC < 3.010
  Thrombocytopenia, TPC < 1308
Cytopenia leading to discontinuation or decreased DMARD12
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.