INTESTINAL LYMPHANGIECTASIA - A PROTEIN-LOSING ENTEROPATHY WITH HYPOGAMMAGLOBULINEMIA LYMPHOCYTOPENIA AND IMPAIRED HOMOGRAFT REJECTION
INTESTINAL LYMPHANGIECTASIA - A PROTEIN-LOSING ENTEROPATHY WITH HYPOGAMMAGLOBULINEMIA LYMPHOCYTOPENIA AND IMPAIRED HOMOGRAFT REJECTION
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DOI：
10.1172/jci105656
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发表时间：
1967-01-01
影响因子：
15.9
通讯作者：
WALDMANN, TA
中科院分区：
文献类型：
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作者：
STROBER, W;WOCHNER, RD;WALDMANN, TA
肠淋巴管扩张症是一种以扩张性肠淋巴管炎、蛋白丢失性肠病、低白蛋白血症和水肿为特征的疾病。本文对18例肠淋巴管扩张症患者的免疫状态进行了研究。通过免疫沉淀法测定IG [免疫球蛋白]G、伊加和IgM的浓度，并使用纯化的放射性碘标记蛋白研究这3种免疫球蛋白的代谢。各免疫球蛋白的血清浓度和全身池均大大降低。每天分解代谢的血管内蛋白池的分数增加到34%的IgG，59%的伊加，和66%的IgM，这些是在对照值的7%，28%，和17%，分别。免疫球蛋白合成率正常或略有增加。在5例Vi和兔热病抗原患者中检测了初级循环抗体应答。在具有Vi抗原的患者中引起的滴度显著低于在对照组中观察到的滴度，而在患者和对照对土拉菌抗原的应答之间没有观察到差异。肠淋巴管扩张患者出现淋巴细胞减少。平均循环淋巴细胞计数为710[±]340/mm 3，对照组为2500 [±] 600/mm 3。通过皮肤试验和皮肤移植研究细胞超敏反应。91%的正常人对4种皮试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。3例二硝基氯苯试验均为阴性反应。最后，所有4例接受同种异体皮肤移植的患者均保留了这些移植物至少12个月。肠淋巴管扩张症患者的免疫功能紊乱似乎是由于淋巴通道紊乱导致免疫球蛋白和淋巴细胞进入胃肠道的损失。然后淋巴细胞耗竭导致皮肤无反应性和同种移植物排斥反应受损。
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 Ig [immunoglobulin]G, IgA, and IgM were measured by immune precipitation and metabolism of these 3 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 immunoglobulins were normal or slightly increased. Primary circulating antibody response was tested in 5 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[plus or minus]340/mm3 in contrast to 2500 [plus or minus] 600/mm3 in controls. Cellular hypersensitivity was studied with skin tests and skin grafts. 91% of normal individuals reacted to at least 1 of the 4 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 3 patients tested with dinitrochlorobenzene had a negative reaction. Finally, all 4 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.