Hull D R, McMillan S A, Rea I M, Boyd N, McMullin M F
Department of Haematology, Royal Victoria Hospital, Belfast.
Ulster Med J. 1996 May;65(1):55-7.
Antibodies to neutrophil cytoplasmic antigens (ANCA) are good serological markers for patients with mainly vasculitic conditions. Two main types of ANCAs have been detected, the first termed cytoplasmic antineutrophil cytoplasmic antibody (cANCA) are mainly associated with patients with Wegener's granulomatosis, the other termed perinuclear antineutrophil cytoplasmic antibody (pANCA) are mainly associated with patients with renal vasculitis, rheumatic and collagen disorders. These antibodies are against various constituents of neutrophil granules. In patients with myelodysplasia, defects in normal granulocyte development are seen. We report a series of twelve patients with myelodysplasia of whom at least four showed a low titre and one a high titre of pANCA. Two of these patients also had demonstrable activity against myeloperoxidase (MPO). None of these patients had any evidence of systemic or cutaneous vasculitis or of any autoimmune disorder. There was no pANCA positivity in an age matched control group.
抗中性粒细胞胞浆抗原（ANCA）抗体是主要患有血管炎病症患者的良好血清学标志物。已检测到两种主要类型的ANCA，第一种称为胞浆抗中性粒细胞胞浆抗体（cANCA），主要与韦格纳肉芽肿病患者相关，另一种称为核周抗中性粒细胞胞浆抗体（pANCA），主要与肾血管炎、风湿和胶原病患者相关。这些抗体针对中性粒细胞颗粒的各种成分。在骨髓增生异常综合征患者中，可见正常粒细胞发育缺陷。我们报告了一系列12例骨髓增生异常综合征患者，其中至少4例显示低滴度pANCA，1例显示高滴度pANCA。这些患者中有2例还具有针对髓过氧化物酶（MPO）的可证实活性。这些患者均无任何系统性或皮肤血管炎或任何自身免疫性疾病的证据。在年龄匹配的对照组中未发现pANCA阳性。
