Papadaki H A, Chatzivassili A, Stefanaki K, Koumaki V, Kanavaros P, Eliopoulos G D
Department of Hematology, University of Crete School of Medicine, University Hospital of Heraklion, Greece.
Ann Hematol. 2000 Oct;79(10):563-70. doi: 10.1007/s002770000207.
This report describes the morphologically defined myeloid cell compartments, lymphocyte subpopulations, and histological findings of bone marrow in 38 patients with nonimmune chronic idiopathic neutropenia of adults (NI-CINA) and in 14 controls. We found that patients had a striking shift to the left of the granulocytic series due to both an increased proportion of proliferating cells and a reduced proportion of maturating cells compared with controls (P<0.001 and P<0.001, respectively). Individual proportions of these cells strongly correlated with the number of circulating neutrophils (r = -0.462, P < 0.01 and r = 0.495, P<0.01, respectively). However, in the great majority of patients (78.9%), no significant changes in marrow cellularity or the myeloid to erythroid cell ratio could be demonstrated. Patients also had increased proportions of CD19+B cells, CD20+B cells, and plasma cells with polytypic expression relative to controls (P < 0.02, P< 0.01, and P< 0.001, respectively). Individual values of plasma cells were inversely correlated with the number of blood neutrophils (r=-0.414, P<0.01). Dispersed bcl-2+lymphocytic aggregates without germinal centers were seen in about one-third of the patients. T cells and natural killer (NK) cells did not show any significant change. Patients had increased proportions of CD57+, CD16+, and HLA-DR+ cells and, in a few cases, increased proportions of histiocytes and eosinophils. CD45RO+ cells were reduced only in patients with pronounced neutropenia. Expression of p53 protein has not been detected in any cell population. With the exception of some megaloblastoid features of erythroid lineage seen in two patients and the presence of some micromegacaryocytes seen in two others, no significant morphological abnormalities were noted. All of these findings are consistent with our previously reported suggestion for the possible existence of an underlying low-grade chronic inflammatory process in NI-CINA patients, which may be involved in the pathogenesis of neutropenia in the affected subjects.
本报告描述了38例成年非免疫性慢性特发性中性粒细胞减少症（NI-CINA）患者和14例对照者骨髓中形态学定义的髓细胞区室、淋巴细胞亚群及组织学表现。我们发现，与对照组相比，患者粒细胞系显著左移，原因是增殖细胞比例增加以及成熟细胞比例降低（分别为P<0.001和P<0.001）。这些细胞的个体比例与循环中性粒细胞数量密切相关（分别为r = -0.462，P < 0.01和r = 0.495，P<0.01）。然而，绝大多数患者（78.9%）的骨髓细胞密度或髓系与红系细胞比例无显著变化。相对于对照组，患者的CD19+B细胞、CD20+B细胞及多型表达浆细胞比例也增加（分别为P < 0.02，P< 0.01和P< 0.001）。浆细胞个体值与血液中性粒细胞数量呈负相关（r=-0.414，P<0.01）。约三分之一的患者可见散在的无生发中心的bcl-2+淋巴细胞聚集物。T细胞和自然杀伤（NK）细胞未见任何显著变化。患者的CD57+、CD16+及HLA-DR+细胞比例增加，并在少数病例中可见组织细胞和嗜酸性粒细胞比例增加。仅在严重中性粒细胞减少的患者中CD45RO+细胞减少。在任何细胞群体中均未检测到p53蛋白表达。除两名患者出现红系的一些巨幼样特征以及另外两名患者存在一些微小巨核细胞外，未发现明显形态学异常。所有这些发现均与我们之前报道的关于NI-CINA患者可能存在潜在低度慢性炎症过程的观点一致，该过程可能参与了受累个体中性粒细胞减少的发病机制。
