Mezger J, Malfertheiner P, Glasmacher A, Breuer T H, Fischer H P, Meybehm M, Kipnowski H J, Sauerbruch T
Medizinische Klinik und Poliklinik-Allgemeine Innere Medizin und Pathologisches Institut, Universität Bonn sowie Innere Medizin-Rheumatologie, Brüderkrankenhaus St. Petrus, Bonn.
Z Gastroenterol. 1996 Oct;34(10):699-703.
A 22-year-old Libyan patient suffering from chronic diarrhea presented with an alpha-heavy chain paraprotein and a lympho-plasmacellular lymphoma infiltration of the duodenal mucosa. These findings supported the diagnosis of "immunoproliferative small intestinal disease" (IPSID). In this disease, that occurs almost solely in countries with low socioeconomic status, a diffuse infiltration of small intestinal mucosa by neoplastic lymphoid cells causes chronic malabsorption. About 65% of patients exhibit a paraprotein in serum, urine or jejunal juice that consists of the heavy chain of immunoglobulin A (alpha-heavy chain). In advanced stages, IPSID resembles histologically and clinically high grade lymphoma: some patients develop masses in the gut wall, an abdominal lymphadenopathy and involvement of other organs including bone marrow. The disease is believed to be triggered by a chronic infectious antigenic stimulus. Thus, in early stages in some patients cure may be achieved by antibiotic therapy alone. In advanced disease, chemotherapy including anthracyclins is necessary.
一名患有慢性腹泻的22岁利比亚患者出现α重链副蛋白以及十二指肠黏膜的淋巴浆细胞性淋巴瘤浸润。这些发现支持了“免疫增殖性小肠疾病”（IPSID）的诊断。在这种几乎仅发生于社会经济地位较低国家的疾病中，肿瘤性淋巴细胞对小肠黏膜的弥漫性浸润会导致慢性吸收不良。约65%的患者在血清、尿液或空肠液中出现由免疫球蛋白A重链（α重链）组成的副蛋白。在疾病晚期，IPSID在组织学和临床上类似于高级别淋巴瘤：一些患者会在肠壁形成肿块、出现腹部淋巴结病并累及包括骨髓在内的其他器官。该疾病被认为是由慢性感染性抗原刺激引发的。因此，在某些患者的早期阶段，仅通过抗生素治疗就可能治愈。在晚期疾病中，包括蒽环类药物在内的化疗是必要的。
