Nash H, Binns G F, Clarkson A R, Beare T H
Aust N Z J Med. 1978 Apr;8(2):184-8. doi: 10.1111/j.1445-5994.1978.tb04509.x.
A case of concomitant cyclical neutropaenia and IgA nephropathy, a previously undescribed combination is reported. The patient has recurrent aphthous ulceration, and haematuria occurs with these episodes. The diagnosis of cyclical neutropaenia was based on the clinical features and serial peripheral blood studies, and a renal biopsy studied by light, electron and immunofluorescence microscopy in conjunction with the clinical features established the diagnosis of IgA nephropathy. Elevation of serum immunoglobulin, with a disproportionate elevation of IgA was found. The features of this case emphasise the importance of infection as an initiating event in the pathogenesis of IgA nephropathy, and they provide further evidence for the formation of poorly soluble immune complexes as a likely pathogenetic mechanism.
报告了一例同时患有周期性中性粒细胞减少症和IgA肾病的病例,这是一种此前未被描述过的组合。患者反复出现口腔溃疡,且在这些发作期间出现血尿。周期性中性粒细胞减少症的诊断基于临床特征和系列外周血研究,结合临床特征,通过光镜、电镜和免疫荧光显微镜对肾活检组织进行研究,确诊为IgA肾病。发现血清免疫球蛋白升高,其中IgA升高尤为明显。该病例的特征强调了感染作为IgA肾病发病机制中起始事件的重要性,并为形成难溶性免疫复合物作为可能的发病机制提供了进一步证据。