Strober W, Wochner R D, Carbone P P, Waldmann T A
J Clin Invest. 1967 Oct;46(10):1643-56. doi: 10.1172/JCI105656.
Intestinal lymphangiectasia is a disease characterized by dilated intestinal lymphatics, protein-losing enteropathy, hypoalbuminemia, and edema. The immunologic status of 18 patients with intestinal lymphangiectasia was studied. Concentrations of IgG, IgA, and IgM were measured by immune precipitation and metabolism of these three immunoglobulins was studied using purified radioiodinated proteins. The serum concentration and total body pool of each immunoglobin were greatly reduced. The fraction of the intravascular protein pool catabolized per day was increased to 34% for IgG, 59% for IgA, and 66% for IgM; these are in contrast with control values of 7%, 28%, and 17%, respectively. Synthetic rates of the immunoglobulins were normal or slightly increased. Primary circulating antibody response was tested in five patients with Vi and tularemia antigens. Titers elicited in patients with the Vi antigen were significantly lower than those seen in a control group, whereas no difference was seen between patient and control responses to the tularemia antigen. Lymphocytopenia was noted in patients with intestinal lymphangiectasia. The mean circulating lymphocyte count was 710 +/- 340/mm(3) in contrast to 2500 +/- 600/mm(3) in controls. Cellular hypersensitivity was studied with skin tests and skin grafts. 91% of normal individuals reacted to at least one of the four skin test antigens: purified protein derivative, mumps, Trichophyton, and Candida albicans; in contrast, only 17% of patients with intestinal lymphangiectasia had a positive reaction. Each of three patients tested with dinitrochlorobenzene had a negative reaction. Finally, all four patients who received skin homografts have retained these grafts for at least 12 months. The immunological disorders in patients with intestinal lymphangiectasia appear to result from loss of immunoglobulins and lymphocytes into the gastrointestinal tract secondary to disorders of lymphatic channels. Lymphocyte depletion then leads to skin anergy and impaired homograft rejection.
肠淋巴管扩张症是一种以肠淋巴管扩张、蛋白丢失性肠病、低白蛋白血症和水肿为特征的疾病。对18例肠淋巴管扩张症患者的免疫状态进行了研究。采用免疫沉淀法测定IgG、IgA和IgM的浓度，并使用纯化的放射性碘化蛋白研究这三种免疫球蛋白的代谢。每种免疫球蛋白的血清浓度和全身总量均大幅降低。每天分解代谢的血管内蛋白池比例，IgG增至34%，IgA增至59%，IgM增至66%；相比之下，对照组的值分别为7%、28%和17%。免疫球蛋白的合成速率正常或略有增加。对5例感染Vi抗原和土拉菌病抗原的患者进行了初次循环抗体反应检测。Vi抗原患者产生的抗体滴度明显低于对照组，而患者和对照组对土拉菌病抗原的反应无差异。肠淋巴管扩张症患者存在淋巴细胞减少。患者循环淋巴细胞平均计数为710±340/mm³，而对照组为2500±600/mm³。通过皮肤试验和皮肤移植研究细胞超敏反应。91%的正常个体对四种皮肤试验抗原中的至少一种有反应：纯化蛋白衍生物、腮腺炎、毛癣菌和白色念珠菌；相比之下，只有17%的肠淋巴管扩张症患者有阳性反应。用二硝基氯苯检测的3例患者均呈阴性反应。最后，接受皮肤同种异体移植的4例患者均保留这些移植片至少12个月。肠淋巴管扩张症患者的免疫紊乱似乎是由于淋巴管疾病继发免疫球蛋白和淋巴细胞丢失到胃肠道所致。淋巴细胞耗竭进而导致皮肤无反应性和同种异体移植排斥受损。
