Mayer L, Fu S M, Cunningham-Rundles C, Kunkel H G
J Clin Invest. 1984 Dec;74(6):2115-20. doi: 10.1172/JCI111636.
B cells from 25 patients with common variable immunodeficiency (CVI) were tested for their ability to differentiate under the influence of B cell differentiation factors (BCDF), derived from T cell hybridomas or T cell clones. 11 patients generated Ig plaque-forming cells in the range comparable to that of normal controls with supernatant from the T cell hybrid MOP 1L. With various hybrid or clone supernatants, differing response patterns emerged. Four patients who failed to respond to MOP 1L responded to T cell clone supernatant RAC. Another who failed to respond to both MOP 1L and RAC responded to T cell hybrid supernatant MTP 7. These results indicate that these supernatants contain different BCDFs and suggest heterogeneity in the differentiation states of B cells in CVI. In addition, three patients demonstrated exaggerated responses to BCDF, and evidence was obtained from B cells of these patients for increased BCDF receptor density. Thus, the accumulated evidence indicates that T cell defects may be a primary pathogenetic mechanism in common variable immunodeficiency, and purified BCDF may be of therapeutic value.
对25例常见可变免疫缺陷(CVI)患者的B细胞进行检测,以观察其在源自T细胞杂交瘤或T细胞克隆的B细胞分化因子(BCDF)影响下的分化能力。11例患者在使用T细胞杂交瘤MOP 1L的上清液时,产生的Ig斑块形成细胞数量与正常对照相当。使用各种杂交瘤或克隆上清液时,出现了不同的反应模式。4例对MOP 1L无反应的患者对T细胞克隆上清液RAC有反应。另1例对MOP 1L和RAC均无反应的患者对T细胞杂交瘤上清液MTP 7有反应。这些结果表明,这些上清液含有不同的BCDF,并提示CVI中B细胞分化状态存在异质性。此外,3例患者对BCDF表现出过度反应,并且从这些患者的B细胞中获得证据表明BCDF受体密度增加。因此,积累的证据表明,T细胞缺陷可能是常见可变免疫缺陷的主要发病机制,纯化的BCDF可能具有治疗价值。