Duggan-Keen M F, Bird A G, Bird P, Smith S W, Givan A L, Calvert J E
Division of Immunology, School of Pathological Sciences, University of Newcastle upon Tyne.
Dis Markers. 1990 Mar-Apr;8(2):69-83.
Lymphocyte function and cell surface phenotype were examined in fifteen patients with late onset hypogammaglobulinaemia. The percentage of surface immunoglobulin-positive B cells in fourteen of the fifteen patients was in the normal range. Patients' B cells expressed MHC class II antigens at normal levels. For one patient, there was relatively high sIgD and low sIgM expression on B cells; the rest of the patients did not differ from controls in surface immunoglobulin density. The proportion of B cells positive for CD5 in patients was comparable to normal controls, and considerably less than in cord blood. However, the pattern of immunoglobulin isotype secretion in vitro by patients' B cells closely paralleled responses of cord blood B cells. Spontaneous secretion of IgM and IgG by patients' B cells was very low. Following polyclonal activation in the presence of autologous T cells, cells from thirteen patients secreted IgM within the normal range in response to at least one activator. The response of patients' purified B cells to IL-2 and gamma-IFN was variable. For four of six tested, B cells cultured with IL-2 and gamma-IFN together with polyclonal activators secreted normal levels of IgM. B cells from the other two patients secreted little or no IgM in response to these cytokines. For fourteen patients, IgG secretion following polyclonal activation remained low both when B cells were cultured with T cells or with a combination of IL-2 and gamma-IFN. IgG subclass imbalance was seen in one patient, whose cells secreted an unusually high proportion of IgG3, and undetectable IgG2 and IgG4; this pattern was consistent whether T cell help was provided by autologous or allogeneic T cells. Similarly purified B cells from this patient showed deficient IgG2 and IgG4 production in response to IL-2 and gamma-IFN.
对15例迟发性低丙种球蛋白血症患者的淋巴细胞功能和细胞表面表型进行了检测。15例患者中有14例表面免疫球蛋白阳性B细胞的百分比在正常范围内。患者的B细胞以正常水平表达MHC II类抗原。有1例患者的B细胞上sIgD表达相对较高而sIgM表达较低；其余患者的表面免疫球蛋白密度与对照组无差异。患者中CD5阳性B细胞的比例与正常对照组相当，且远低于脐血中的比例。然而，患者B细胞体外免疫球蛋白同种型分泌模式与脐血B细胞的反应非常相似。患者B细胞自发分泌IgM和IgG的水平非常低。在自体T细胞存在下进行多克隆激活后，13例患者的细胞对至少一种激活剂的反应分泌出正常范围内的IgM。患者纯化B细胞对IL-2和γ-干扰素的反应各不相同。在6例检测的患者中，有4例B细胞与IL-2和γ-干扰素以及多克隆激活剂一起培养时分泌正常水平的IgM。另外2例患者的B细胞对这些细胞因子几乎不分泌或不分泌IgM。对于14例患者，当B细胞与T细胞或与IL-2和γ-干扰素联合培养时，多克隆激活后的IgG分泌仍然很低。在1例患者中观察到IgG亚类失衡，其细胞分泌异常高比例的IgG3，而IgG2和IgG4检测不到；无论自体还是异体T细胞提供T细胞辅助，这种模式都是一致的。同样，该患者纯化的B细胞对IL-2和γ-干扰素的反应显示IgG2和IgG4产生不足。
