Pastorelli G, Roncarolo M G, Peronne C, Tovo P A, de Vries J E
Department of Paediatrics, University of Turin, Italy.
Clin Exp Immunol. 1990 Oct;82(1):120-7. doi: 10.1111/j.1365-2249.1990.tb05414.x.
Interleukin-4 (IL-4) has been shown to induce IgE synthesis by peripheral blood mononuclear cells (PBMC) of normal donors in vitro. However, induction of PBMC of patients with common variable immunodeficiency (CVI) with IL-4 resulted in IgE production in only two out of eight cases tested. PBMC of the first patient that produced IgE in response to IL-4 also secreted normal levels of IL-4 upon activation. PBMC of the second patient secreted very low levels of IL-4 in vitro which may account for the very low serum IgE levels in this patient. Of the other six patients who had very low serum IgE levels and whose PBMC failed to produce IgE in response to IL-4 in vitro, five did not secrete IL-4 upon in vitro activation. The capacity of the T cells to produce IL-4 was intact in the sixth patient. Collectively our data indicate that PBMC of the majority of patients with CVI are defective since they failed to respond appropriately to IL-4 and they failed to produce IL-4, contributing to the view that CVI is a heterogeneous disorder in which a variety of T and B cell defects occur.
白细胞介素-4(IL-4)已被证明可在体外诱导正常供体外周血单个核细胞(PBMC)合成IgE。然而,用IL-4诱导常见可变免疫缺陷(CVI)患者的PBMC时,在8例受测病例中只有2例产生了IgE。首例对IL-4产生反应并产生IgE的患者的PBMC在激活后也分泌正常水平的IL-4。第二例患者的PBMC在体外分泌极低水平的IL-4,这可能是该患者血清IgE水平极低的原因。在其他6例血清IgE水平极低且其PBMC在体外对IL-4无反应而未能产生IgE的患者中,有5例在体外激活时不分泌IL-4。第六例患者的T细胞产生IL-4的能力是完整的。我们的数据总体表明,大多数CVI患者的PBMC存在缺陷,因为它们对IL-4没有适当反应且未能产生IL-4,这支持了CVI是一种异质性疾病的观点,其中存在多种T细胞和B细胞缺陷。