Antibodies to neutrophil cytoplasmic antigens (ANCA) are good serologi
cal markers for patients with mainly vasculitic conditions. Two main t
ypes of ANCAs have been detected the first termed cytoplasmic antineut
rophil cytoplasmic antibody (cANCA) are mainly associated with patient
s with Wegener's granulomatosis, the other termed perinuclear antineut
rophil cytoplasmic antibody (pANCA) are mainly associated with patient
s with renal vasculitis, rheumatic and collagen disorders, These antib
odies are against various constituents of neutrophil granules. In pati
ents with myelodysplasia, defects in normal granulocyte development ar
e seen. We report a series of twelve patients with myelodysplasia of w
hom at least four showed a low titre and one a high titre of pANCA. Tw
o orf these patients also had demonstrable activity against myeloperox
idase (MPO). None of these patients had any evidence of systemic or cu
taneous vasculitis or of any autoimmune disorder. There was no pANCA p
ositivity in an age matched control group.