Hibi T, Asakura H, Kobayashi K, Munakata Y, Kano S, Tsuchiya M, Teramoto T, Uematsu Y
Gut. 1982 May;23(5):422-7. doi: 10.1136/gut.23.5.422.
Ultrastructural and immunohistochemical studies of the small intestine are described in a Japanese patient with alpha heavy chain disease who had a history of colonic ulcers. Endoscopic examinations revealed multiple gastric erosion, duodenal ulcer, and a thickened, cobblestone-like pattern composed of small nodules in the duodenum and jejunum, which was similar in appearance to Crohn's disease. An electron microscopic study showed that the numerous, infiltrated cells in the jejunal lamina propria were matured plasma cells with atypical structure of the organelles. These plasma cells had alpha heavy chain protein devoid of light chain. Although secretory component was demonstrated normally in the epithelial cells by immunofluorescent methods, no association of this component with alpha heavy chain protein could be observed in any of the plasma and epithelial cells of this case. These facts suggest the absence of secretory alpha chain or secretory IgA, and a deficiency of the mucosal secretory immune system in this patient.
对一名有结肠溃疡病史的日本α重链病患者的小肠进行了超微结构和免疫组织化学研究。内镜检查发现多处胃糜烂、十二指肠溃疡，十二指肠和空肠有由小结节组成的增厚的鹅卵石样图案，外观与克罗恩病相似。电子显微镜研究显示，空肠固有层中大量浸润细胞为细胞器结构异常的成熟浆细胞。这些浆细胞含有不含轻链的α重链蛋白。尽管通过免疫荧光法在上皮细胞中正常显示了分泌成分，但在该病例的任何浆细胞和上皮细胞中均未观察到该成分与α重链蛋白的关联。这些事实表明该患者缺乏分泌性α链或分泌性IgA，且黏膜分泌免疫系统存在缺陷。
