Rodeck U, Kuwert E, Ohnhaus E
Monatsschr Kinderheilkd. 1984 Apr;132(4):238-9.
A 25-year-old patient presented with hypogammaglobulinemia diagnosed at the age of 7, causing recurrent infections of the gastrointestinal and bronchial tracts. He was treated with gammaglobulins and infusions of fresh human plasma. At admission he had an exacerbation of his chronic enteric symptoms. X-ray investigation and coloscopy showed mucosal changes as in enteritis regionalis . A biopsy of rectal mucosa showed accumulations of amyloid. Routine examination of humoral and cellular immune parameters revealed a pattern characteristic for common variable immunodeficiencies. Analysis of T-cell subsets with monoclonal antibodies of the OKT series showed unusually high relative numbers of OKT8+ cells (suppressor-T-lymphocytes) and a comparatively low number of OKT4+ cells (helper-T-lymphocytes). Suppressor-T-cells exert inhibitory effects on B-lymphocyte function (i.e. antibody production). The numerical excess of T-lymphocytes with suppressive properties could be a pathogenetically important factor with immunotherapeutic implications in a number of non-classifiable immunodeficiency syndromes.
一名25岁患者,7岁时被诊断为低丙种球蛋白血症,导致胃肠道和支气管反复感染。他接受了丙种球蛋白和新鲜人血浆输注治疗。入院时,他的慢性肠道症状加重。X线检查和结肠镜检查显示黏膜改变,类似局限性肠炎。直肠黏膜活检显示有淀粉样物质沉积。体液和细胞免疫参数的常规检查显示出常见可变免疫缺陷的特征性模式。用OKT系列单克隆抗体分析T细胞亚群显示,OKT8 +细胞(抑制性T淋巴细胞)的相对数量异常高,而OKT4 +细胞(辅助性T淋巴细胞)的数量相对较低。抑制性T细胞对B淋巴细胞功能(即抗体产生)发挥抑制作用。具有抑制特性的T淋巴细胞数量过多可能是许多不可分类免疫缺陷综合征发病机制中的一个重要因素,具有免疫治疗意义。