Papadaki H A, Eliopoulos D G, Ponticoglou C, Eliopoulos G D
Department of Hematology, University of Crete School of Medicine, University Hospital of Heraklion, Greece.
Int J Hematol. 2001 Apr;73(3):339-45. doi: 10.1007/BF02981959.
This study describes the frequency of monoclonal gammopathy of undetermined significance (MGUS) and the changes in some inflammation-related serum proteins in 157 patients with nonimmune chronic idiopathic neutropenia syndrome (NI-CINS). Of these patients, 42 had pronounced neutropenia with neutrophil counts < 1500/microL, and 115 had mild neutropenia with neutrophil counts ranging from 1500 to 2499/microL. Sixty-six volunteers served as healthy control subjects and 157 age- and sex-matched patients hospitalized for nonmalignant diseases served as patient control subjects. We found that 28.6% of patients with pronounced neutropenia and 14.8% of patients with mild neutropenia had increased serum gamma globulins (above the 95% confidence limit of values of the control subjects). In the group of patients with pronounced neutropenia, 30.9% had increased immunoglobulin (Ig)G values and 23.8% had increased IgA values. In the group of patients with mild neutropenia, 17.4% had increased IgG values and 21.7% had increased IgA values. IgG and IgA values strongly correlated with the neutrophil count. No changes in serum IgM were found. Three of 42 patients with pronounced neutropenia (7.14%) and 3 of 115 patients with mild neutropenia (2.61%) had serum immunofixation tests which showed a small monoclonal spike--4 were IgG-kappa type, 1 was IgG-lambda type, and 1 was IgA-kappa type. None of the healthy or patient control subjects had any evidence of MGUS. No significant changes in the amount of monoclonal spikes were documented during an 18- to 143-month follow-up (median, 58 months). Except for significantly increased alpha1-antitrypsin levels, there were no significant differences in the levels of acute-phase proteins studied between the study patients and the control subjects. These findings are consistent with our previous report suggesting the possible existence of an unrecognized low-grade chronic inflammation in patients with NI-CINS, which may be involved in the pathogenesis of neutropenia in the affected subjects.
本研究描述了157例非免疫性慢性特发性中性粒细胞减少综合征（NI-CINS）患者意义未明的单克隆丙种球蛋白病（MGUS）的发生率以及一些炎症相关血清蛋白的变化。在这些患者中，42例有明显中性粒细胞减少，中性粒细胞计数<1500/μL，115例有轻度中性粒细胞减少，中性粒细胞计数在1500至2499/μL之间。66名志愿者作为健康对照，157名年龄和性别匹配的因非恶性疾病住院的患者作为患者对照。我们发现，28.6%的明显中性粒细胞减少患者和14.8%的轻度中性粒细胞减少患者血清γ球蛋白升高（高于对照受试者值的95%置信限）。在明显中性粒细胞减少的患者组中，30.9%的患者IgG值升高，23.8%的患者IgA值升高。在轻度中性粒细胞减少的患者组中，17.4%的患者IgG值升高，21.7%的患者IgA值升高。IgG和IgA值与中性粒细胞计数密切相关。未发现血清IgM有变化。42例明显中性粒细胞减少的患者中有3例（7.14%），115例轻度中性粒细胞减少的患者中有3例（2.61%）血清免疫固定试验显示有小的单克隆峰——4例为IgG-κ型，1例为IgG-λ型，1例为IgA-κ型。健康对照或患者对照中均无MGUS的证据。在18至143个月的随访（中位数为58个月）期间，未记录到单克隆峰数量的显著变化。除α1-抗胰蛋白酶水平显著升高外，研究患者与对照受试者之间所研究的急性期蛋白水平无显著差异。这些发现与我们之前的报告一致，提示NI-CINS患者可能存在未被认识的低度慢性炎症，这可能参与了受累受试者中性粒细胞减少的发病机制。
