Miyachi H, Nakamura Y, Shimizu H, Watanabe K, Arimori S, Ando Y
Department of Clinical Pathology, Tokai University School of Medicine, Kanagawa, Japan.
Int J Hematol. 1992 Oct;56(2):113-8.
Using a methylcellulose culture system, we studied the effects of recombinant human interleukin-3 (IL-3), recombinant human granulocyte-macrophage colony-stimulating factor (GM-CSF), and recombinant human granulocyte colony-stimulating factor (G-CSF) on the growth of myeloid progenitor cells (CFU-C) from an adult patient with congenital neutropenia. The moderate clinical course and the maturation arrest at blast-promyelocyte stage in the marrow differentiated this patient from those described as having Kostmann-type congenital neutropenia. CFU-C growth in bone marrow cells from the patient responded to IL-3 normally in a dose-dependent manner. GM-CSF stimulated only macrophage colony formation in a dose-dependent manner comparable to that in normal subjects. Neither GM-CSF nor G-CSF stimulated any significant granulocyte colony formation. This evidence suggests that the hematopoietic progenitor cells in this patient had the potential for developing CFU-C with IL-3, and that the neutropenia in this patient could be a result of an intrinsic defect in myelopoiesis along a granulocytic pathway responsive to GM-CSF or G-CSF.
我们使用甲基纤维素培养系统，研究了重组人白细胞介素-3（IL-3）、重组人粒细胞-巨噬细胞集落刺激因子（GM-CSF）和重组人粒细胞集落刺激因子（G-CSF）对一名先天性中性粒细胞减少症成年患者髓系祖细胞（CFU-C）生长的影响。该患者的临床病程中等，骨髓中原始早幼粒细胞阶段出现成熟停滞，这使其与那些被描述为患有 Kostmann 型先天性中性粒细胞减少症的患者有所不同。该患者骨髓细胞中的 CFU-C 生长对 IL-3 呈正常的剂量依赖性反应。GM-CSF 仅以与正常受试者相当的剂量依赖性方式刺激巨噬细胞集落形成。GM-CSF 和 G-CSF 均未刺激任何显著的粒细胞集落形成。这一证据表明，该患者的造血祖细胞具有在 IL-3 作用下发育为 CFU-C 的潜力，并且该患者的中性粒细胞减少可能是由于沿对 GM-CSF 或 G-CSF 有反应的粒细胞生成途径存在内在缺陷所致。
