Logue G L, Shastri K A, Laughlin M, Shimm D S, Ziolkowski L M, Iglehart J L
Department of Medicine, State University of New York, Buffalo.
Am J Med. 1991 Feb;90(2):211-6.
The present study was done to evaluate the clinical characteristics of a large series of adult patients with chronic idiopathic neutropenia, and correlate the presence of antineutrophil antibodies, their class (IgG or IgM), and their ability to fix complement with clinical parameters, including other hemocytopenias, splenomegaly, and infections.
One hundred twenty-one adult patients with chronic idiopathic neutropenia were studied. Serum neutrophil-binding antibodies were measured using paraformaldehyde-fixed granulocytes (PFGs) from normal volunteers as target cells. 125I-labeled staphylococcal protein A was used to detect IgG antibodies while IgM antibodies were detected by using 125I-labeled mouse monoclonal anti-IgM antibody. Sera containing antineutrophil antibodies were tested for their ability to fix complement on donor PFGs by using 125I-labeled monoclonal antibody to the third component of complement.
Of the 121 patients with chronic idiopathic neutropenia, 71 patients had isolated neutropenia, while 50 had neutropenia combined with either anemia and/or thrombocytopenia. Among the 71 patients with isolated neutropenia, there were 51 females (72%), compared with 28 females (56%) among the 50 patients with combined hemocytopenias (p = 0.083). Patients with multiple hemocytopenias were significantly older (p less than 0.01), were more likely to demonstrate splenomegaly (p = 0.001), and may have had more infectious complications. From all the patients, 36% of sera were shown to have antineutrophil antibodies, with a non-significant trend for these to be found more frequently in patients with multiple hemocytopenias. Sera with mixed IgG-IgM antineutrophil antibodies were significantly more likely to fix complement than those with isolated IgG or IgM antibodies, and among the patients with antineutrophil antibodies, complement-fixing antibodies were significantly associated with multiple hemocytopenias. Splenomegaly was significantly associated both with antineutrophil antibodies (p = 0.008) and with infections (p = 0.007). Antineutrophil antibodies were not associated with infections.
Approximately one third of adult patients with idiopathic neutropenia have IgG and/or IgM antineutrophil antibodies demonstrable in their serum. There is a subset of patients with idiopathic neutropenia with multiple hemocytopenias who tend to be older, less likely to show female predominance, more likely to have splenomegaly and infections, and more likely to have antineutrophil antibodies, especially mixed IgG-IgM and complement-fixing antibodies.
本研究旨在评估一大组成年慢性特发性中性粒细胞减少症患者的临床特征，并将抗中性粒细胞抗体的存在、其类别（IgG或IgM）以及其补体结合能力与临床参数相关联，这些临床参数包括其他血细胞减少症、脾肿大和感染。
对121例成年慢性特发性中性粒细胞减少症患者进行了研究。使用来自正常志愿者的经多聚甲醛固定的粒细胞（PFG）作为靶细胞来检测血清中性粒细胞结合抗体。使用125I标记的葡萄球菌蛋白A检测IgG抗体，而使用125I标记的小鼠单克隆抗IgM抗体检测IgM抗体。通过使用125I标记的补体第三成分单克隆抗体，检测含有抗中性粒细胞抗体的血清在供体PFG上的补体结合能力。
在121例慢性特发性中性粒细胞减少症患者中，71例患者为单纯性中性粒细胞减少，而50例患者的中性粒细胞减少合并贫血和/或血小板减少。在71例单纯性中性粒细胞减少患者中，有51例女性（72%），而在50例合并血细胞减少的患者中有28例女性（56%）（p = 0.083）。合并多种血细胞减少的患者年龄显著更大（p < 0.01），更有可能出现脾肿大（p = 0.001），并且可能有更多的感染并发症。在所有患者中，36%的血清显示有抗中性粒细胞抗体，在合并多种血细胞减少的患者中这些抗体更频繁出现，但差异无统计学意义。含有IgG-IgM混合抗中性粒细胞抗体的血清比含有单纯IgG或IgM抗体的血清更有可能结合补体，并且在有抗中性粒细胞抗体的患者中，补体结合抗体与多种血细胞减少显著相关。脾肿大与抗中性粒细胞抗体（p = 0.008）和感染（p = 0.007）均显著相关。抗中性粒细胞抗体与感染无关。
大约三分之一的成年特发性中性粒细胞减少症患者血清中可检测到IgG和/或IgM抗中性粒细胞抗体。有一部分特发性中性粒细胞减少症合并多种血细胞减少的患者往往年龄较大，女性优势不明显，更有可能出现脾肿大和感染，并且更有可能有抗中性粒细胞抗体，尤其是IgG-IgM混合抗体和补体结合抗体。
