Parmley R T, Crist W M, Ragab A H, Boxer L A, Malluh A, Findley H
J Pediatr. 1981 Feb;98(2):207-12. doi: 10.1016/s0022-3476(81)80636-1.
Chronic benign neutropenia of childhood is a heterogeneous disorder. This study describes two severely neutropenic children with a benign clinical course and the unique bone marrow finding of macrophage engulfment of band and segmented neutrophils. Phagocytic vacuoles in the majority of macrophages contained neutrophils in various stages of digestion at both the light and electron microscope level (with as many as four neutrophils observed in single macrophages). Ultrastructural studies demonstrated that the neutrophils were morphologically normal, and apparently viable at the time of phagocytosis. This type of neutrophil phagocytosis was not observed in five other consecutively studied children with CBN. All of these children with CBN had ultrastructurally normal neutrophils, lacked demonstrable antineutrophil antibodies or serum inhibitors to in vitro myelopoiesis, and had normal colony-forming cells and colony-stimulating activity in vitro. Thus despite many similar clinical and laboratory features in children with CBN, only a unique subgroup of these patients demonstrates abnormal neutrophil-macrophage interaction.
儿童慢性良性中性粒细胞减少症是一种异质性疾病。本研究描述了两名严重中性粒细胞减少的儿童,他们临床病程良性,骨髓有独特表现,即巨噬细胞吞噬带状和分叶核中性粒细胞。在光学显微镜和电子显微镜水平下,大多数巨噬细胞中的吞噬泡含有处于不同消化阶段的中性粒细胞(单个巨噬细胞中观察到多达四个中性粒细胞)。超微结构研究表明,中性粒细胞形态正常,在被吞噬时显然仍有活力。在另外连续研究的五名儿童慢性良性中性粒细胞减少症患者中未观察到这种类型的中性粒细胞吞噬现象。所有这些慢性良性中性粒细胞减少症患儿的中性粒细胞超微结构均正常,缺乏可检测到的抗中性粒细胞抗体或体外骨髓生成的血清抑制剂,并且体外集落形成细胞和集落刺激活性正常。因此,尽管慢性良性中性粒细胞减少症患儿有许多相似的临床和实验室特征,但只有这些患者中的一个独特亚组表现出异常的中性粒细胞 - 巨噬细胞相互作用。