Dighiero G
Unité d'Immuno-Hématologie et d'Immuno-Pathologie, Institut Pasteur, Paris, France.
Nouv Rev Fr Hematol (1978). 1988;30(5-6):283-8.
Most CLL cases correspond to proliferation of a B-cell clone characterized by: low amounts of surface Ig (SIg); presence of receptors for mouse red blood cells; expression of a 67 Kd antigenic determinant (CD5) initially described to be a pan-T cell antigen. These peculiar characteristics differentiate the CLL B lymphocyte from B lymphocytes proliferating in other B-cell malignancies. These so-called CD5 + B cells constitute a minority of B cells in adults, although they predominate during foetal life. This latter fact induced a majority of authors to assume that CLL B lymphocytes correspond to proliferation of an immature B-cell clone. However, this hypothesis does not explain the particular high frequency with which hypogammaglobulinemia and autoimmune phenomena directed against blood cell components are found in B-CLL, as well as the difficulty to include CD5 antigen in most differentiation pathways of B-cells. In this work, we shall discuss the available evidence coming from Ly 1-B cells (mouse counterpart of CD5 B cells), indicating that these cells correspond to a separate B lineage. We shall also discuss the evidence for hypogammaglobulinemia being mainly a B cell defect caused by dilution or inhibition of normal CD5 negative B cells and disordered autoimmunity as a consequence of a disturbance of the idiotypic network, which normally antagonizes autoimmune clones.
大多数慢性淋巴细胞白血病（CLL）病例对应于一种B细胞克隆的增殖，其特征为：表面免疫球蛋白（SIg）含量低；存在小鼠红细胞受体；表达一种最初被描述为泛T细胞抗原的67 Kd抗原决定簇（CD5）。这些独特的特征将CLL B淋巴细胞与在其他B细胞恶性肿瘤中增殖的B淋巴细胞区分开来。这些所谓的CD5 + B细胞在成年人中占B细胞的少数，尽管它们在胎儿期占主导地位。后一事实使大多数作者认为CLL B淋巴细胞对应于未成熟B细胞克隆的增殖。然而，这一假设无法解释在B-CLL中发现低丙种球蛋白血症和针对血细胞成分的自身免疫现象的特别高频率，以及在B细胞的大多数分化途径中纳入CD5抗原的困难。在这项工作中，我们将讨论来自Ly 1-B细胞（CD5 B细胞的小鼠对应物）的现有证据，表明这些细胞对应于一个独立的B细胞系。我们还将讨论低丙种球蛋白血症主要是由正常CD5阴性B细胞的稀释或抑制以及由于独特型网络紊乱导致的自身免疫失调引起的B细胞缺陷的证据，独特型网络通常对抗自身免疫克隆。
