Uluhan A, Sager D, Jasin H E
Department of Internal Medicine, University of Arkansas for Medical Sciences, John L. McClellan Veterans Administration Hospital, Little Rock 72205, USA.
J Rheumatol. 1998 Jun;25(6):1205-10.
We describe a 9-year-old white boy with systemic juvenile rheumatoid arthritis (JRA) who developed pancytopenia and hypersplenism at the age of 13 years. He underwent splenectomy and 3 years later he developed Coombs' positive hemolytic anemia, alopecia, juvenile warts, and multiple bacterial infections. At that time, investigations were compatible with severe hypogammaglobulinemia associated with common variable immunodeficiency. Concomitantly with this condition he experienced complete remission of his inflammatory arthritis. Immunologic studies of B and T lymphocyte function showed that the number of circulating T and B lymphocytes were normal, while T cell function was depressed, as evidenced by markedly reduced proliferative responses to mitogens and antigens, and ability to mediate B cell help. In addition, his circulating B cells were unable to secrete IgM or IgG. He also exhibited anergy to intradermal challenge with a battery of common antigens. The literature dealing with this clinical association is reviewed, and possible immunologic mechanisms involved are discussed.
我们描述了一名9岁白人男孩，患有全身型幼年类风湿性关节炎（JRA），13岁时出现全血细胞减少和脾功能亢进。他接受了脾切除术，3年后出现库姆斯试验阳性的溶血性贫血、脱发、青少年疣和多种细菌感染。当时的检查结果与伴有常见可变免疫缺陷的严重低丙种球蛋白血症相符。与此同时，他的炎性关节炎完全缓解。对B和T淋巴细胞功能的免疫学研究表明，循环T和B淋巴细胞数量正常，但T细胞功能低下，这表现为对有丝分裂原和抗原的增殖反应明显降低，以及介导B细胞辅助的能力下降。此外，他循环中的B细胞无法分泌IgM或IgG。他对一系列常见抗原的皮内刺激也表现出无反应性。本文回顾了有关这种临床关联的文献，并讨论了可能涉及的免疫机制。
