Tashiro T, Sato H, Takahashi T, Genda T, Sugitani S, Yoshida T, Funakoshi K, Tukada Y, Narisawa R, Nomoto M
Third Department of Internal Medicine, Niigata University School of Medicine.
Intern Med. 1995 Apr;34(4):255-60. doi: 10.2169/internalmedicine.34.255.
A case of alpha chain disease, involving stomach, small and large intestine, and caecum with poor prognosis is reported. Endoscopic examination revealed gastric erosion, edematous mucosa with enlarged villi of duodenum and jejunum, multiple hyperplastic lymph follicles of terminal ileum and thickening mucosa of caecum. Light microscopy revealed a conspicuous infiltration of plasma cells and lymphocytes in gastric, duodenal, jejunal and caecal lamina propria. Immunohistochemistry demonstrated alpha heavy chain protein devoid of light chain in these plasma cells. The patient developed paralytic ileus and died of septic shock on the 179th hospital day.
报告了一例α链病，累及胃、小肠和大肠以及盲肠，预后不良。内镜检查显示胃黏膜糜烂、十二指肠和空肠黏膜水肿伴绒毛增大、回肠末端多个增生性淋巴滤泡以及盲肠黏膜增厚。光镜检查显示胃、十二指肠、空肠和盲肠固有层有明显的浆细胞和淋巴细胞浸润。免疫组化显示这些浆细胞中存在无轻链的α重链蛋白。患者出现麻痹性肠梗阻，并在住院第179天死于感染性休克。
