Jones B M, Lau Y L, Wong K L
Department of Pathology, Queen Mary Hospital, Hong Kong.
Eur J Pediatr. 1993 May;152(5):409-13. doi: 10.1007/BF01955899.
We report six Chinese boys with hypogammaglobulinaemia. All but one had very low or undetectable circulating B-lymphocytes, two had reversed CD4/CD8 ratios (in one of whom this latter became normal), one had reduced lymphocyte proliferative responses to concanavalin A and pokeweed mitogen and three had deficient responses to OKT3. Generation of antibody-secreting cells in response to pokeweed mitogen was markedly defective in all patients. Co-cultures of purified lymphocyte subsets from the patients with those of normal donors revealed that in addition to B-cell deficiency seen in all patients, two had T-helper cell deficiency and two had T-suppressor cell hyperactivity. One of the latter patients was treated with cimetidine in an attempt to ablate histamine type 2 receptor-bearing suppressor cells: the absolute number of such cells was temporarily reduced but there was no concurrent correction of the functional hyperactivity. These studies point to the variable nature of T-regulatory cell deficiencies in hypogammaglobulinaemia.
我们报告了6例患有低丙种球蛋白血症的中国男孩。除1例之外,其余所有患儿循环B淋巴细胞水平极低或检测不到,2例患儿CD4/CD8比值倒置(其中1例后来恢复正常),1例患儿对刀豆蛋白A和商陆有丝分裂原的淋巴细胞增殖反应降低,3例患儿对OKT3反应不足。所有患者对商陆有丝分裂原产生抗体分泌细胞的能力均明显缺陷。将患者纯化的淋巴细胞亚群与正常供体的淋巴细胞亚群进行共培养发现,除所有患者均存在B细胞缺陷外,2例有辅助性T细胞缺陷,2例有抑制性T细胞活性亢进。后1组中的1例患者接受了西咪替丁治疗,试图消除表达组胺2型受体的抑制细胞:这类细胞的绝对数量暂时减少,但功能性亢进并未同时得到纠正。这些研究表明低丙种球蛋白血症中T调节细胞缺陷具有多变性。