Savige J A, Chang L, Smith C L, Duggan J C
Department of Medicine, Austin Hospital, Melbourne, Vic., Australia.
Aust N Z J Med. 1994 Jun;24(3):282-7. doi: 10.1111/j.1445-5994.1994.tb02173.x.
Anti-neutrophil cytoplasmic antibodies (ANCA) are typically associated with small vessel vasculitides. They are also found in situations where other autoantibodies are common, sometimes after infections and possibly in individuals who have received multiple blood transfusions.
The aim of this study was to determine the incidence of ANCA in a variety of haematological disorders, where these predisposing factors may be at work.
Sera from patients with myelodysplasia (n = 26), acute myeloid leukaemia (AML) (n = 3), and myeloproliferative (n = 25) or lymphoproliferative syndromes (n = 16) were screened for ANCA using a crude neutrophil cytoplasmic extract ELISA and indirect immunofluorescent examination of normal peripheral blood neutrophils. Positive results were confirmed by ELISAs for anti-proteinase 3, anti-myeloperoxidase or anti-elastase antibodies.
ANCA were demonstrated in two patients with myelodysplasia, both with chronic myelomonocytic leukaemia and greater than 5% blasts in the bone marrow. Both of these individuals were infected at the time that ANCA were demonstrated and other autoantibodies were present. One of these individuals had never had evidence of any vasculitis; the other probably developed myelodysplasia after treatment with cyclophosphamide for Wegener's granulomatosis. ANCA were demonstrated in one individual with AML secondary to myelodysplasia. ANCA were also found in a patient with lymphoma in whom autoantibodies against red cells and platelets were already noted. ANCA were demonstrated in one further individual with lymphomatoid granulomatosis, a condition that resembles Wegener's granulomatosis clinically and histologically, but which is treated as a lymphoma. No ANCA were present in any of the patients with myeloproliferative syndromes.
ANCA probably occur secondary to immune dysregulation in myelodysplasia and the lymphoproliferative conditions and they are not necessarily associated with the presence of a vasculitis.
抗中性粒细胞胞浆抗体（ANCA）通常与小血管炎相关。在其他自身抗体常见的情况下也能发现它们，有时在感染后，也可能在接受多次输血的个体中出现。
本研究的目的是确定在各种血液系统疾病中ANCA的发生率，这些疾病中可能存在上述诱发因素。
使用粗制中性粒细胞胞浆提取物酶联免疫吸附测定（ELISA）和正常外周血中性粒细胞的间接免疫荧光检查，对骨髓增生异常综合征患者（n = 26）、急性髓系白血病（AML）患者（n = 3）、骨髓增殖性疾病患者（n = 25）或淋巴细胞增殖性综合征患者（n = 16）的血清进行ANCA筛查。通过抗蛋白酶3、抗髓过氧化物酶或抗弹性蛋白酶抗体的ELISA来确认阳性结果。
在两名骨髓增生异常综合征患者中检测到ANCA，这两名患者均患有慢性粒单核细胞白血病，且骨髓中原始细胞比例大于5%。在检测到ANCA时，这两名患者均受到感染，且存在其他自身抗体。其中一名患者从未有过任何血管炎的证据；另一名患者可能在因韦格纳肉芽肿接受环磷酰胺治疗后发生了骨髓增生异常综合征。在一名继发于骨髓增生异常综合征的AML患者中检测到ANCA。在一名已发现存在抗红细胞和抗血小板自身抗体的淋巴瘤患者中也发现了ANCA。在另一名淋巴瘤样肉芽肿患者中检测到ANCA，该疾病在临床和组织学上类似于韦格纳肉芽肿，但被视为淋巴瘤。骨髓增殖性综合征患者中均未检测到ANCA。
ANCA可能继发于骨髓增生异常综合征和淋巴细胞增殖性疾病中的免疫失调，且不一定与血管炎的存在相关。
