Adamson J W
Clin Haematol. 1984 Jun;13(2):489-502.
The clonal haemopathies have in common the expansion of a neoplastic stem cell which has retained a variable capacity for differentiation to granulocytes, monocytes, erythrocytes, and platelets. Although normal stem cells are present in such patients, their differentiation is somehow suppressed. However, their presence may be demonstrated either by cell culture studies, employing G6PD as the marker in appropriately selected cases, or by their emergence in patients subjected to marrow ablative chemotherapy. Suppression of normal stem cell growth and development does not require a hyperproliferative marrow, and the current data do not support the notion that normal stem cells are simply reduced in frequency by a vast increase in the numbers of neoplastic progenitors. It is likely, therefore, that such suppression involves cellular signals, operating over short distances (perhaps through cell-to-cell contact), which interfere with normal progenitors. Thus, stem cell culture techniques, in combination with cell markers, have provided a direct and informative way to approach the issues of disease pathogenesis, the kinetics of stem cell differentiation, and the interaction of normal and neoplastic cells in patients with clonal haemopathies.
克隆性血液病的共同特点是肿瘤性干细胞的扩增,该干细胞保留了向粒细胞、单核细胞、红细胞和血小板分化的可变能力。虽然这类患者体内存在正常干细胞,但其分化受到某种程度的抑制。然而,可通过细胞培养研究(在适当选择的病例中采用G6PD作为标志物)或通过在接受骨髓清除性化疗的患者中出现正常干细胞来证明其存在。正常干细胞生长和发育的抑制并不需要骨髓增殖亢进,目前的数据也不支持正常干细胞仅仅因肿瘤祖细胞数量大幅增加而频率降低的观点。因此,这种抑制可能涉及短距离作用的细胞信号(可能通过细胞间接触),这些信号会干扰正常祖细胞。因此,干细胞培养技术与细胞标志物相结合,为探讨克隆性血液病患者的疾病发病机制、干细胞分化动力学以及正常细胞与肿瘤细胞的相互作用提供了一种直接且信息丰富的方法。