Broder S, Callihan T R, Jaffe E S, DeVita V T, Strober W, Bartter F C, Waldmann T A
Gastroenterology. 1981 Jan;80(1):166-8.
In 1956 we evaluated a patient who had a debilitating disease of a 2 yr duration, characterized by recurrent vomiting, diarrhea, cachexia, massive edema, hypoproteinemia, and dilated intestinal lymphatics. During our initial evaluation of this patient, we observed that 42% of her circulating protein pool was lost into her gastrointestinal tract daily, whereas normal gastrointesinal loss of protein does not exceed 1.6%. Her disease appeared to represent a classic example of intestinal lymphangiectasia. She was treated symptomatically for 13 yr with essentially no change. In 1969 the patient developed a stage IV diffuse, undifferentiated (non-Burkitt's) malignant lymphoma. Using immunoperoxidase staining, the neoplastic cells were found to contain cytoplasmic IgMKappa, suggesting that the lymphoma had a monoclonal B-cell origin. She was successfully treated with cyclophosphamide, vincristine, and prednisone. Shortly after the initiation of this systemic combination chemotherapy, her serum protein concentration returned to normal, her edema resolved, and she was cured of gastrointestinal symptoms. Moreover, repeat studies revealed that her protein loss had fallen to only 2%. The simultaneous cure of both the intestinal lymphangiectasia and lymphoma with combination chemotherapy suggests new relationships between these conditions as well as new possibilities for the treatment of acquired forms of intestinal lymphangiectasis associated with overwhelming gastrointestinal protein loss.
1956年，我们评估了一位患有持续两年衰弱性疾病的患者，其特征为反复呕吐、腹泻、恶病质、大量水肿、低蛋白血症以及肠道淋巴管扩张。在对该患者进行初步评估期间，我们观察到她每天循环蛋白池的42%会流失到胃肠道，而正常情况下胃肠道的蛋白流失不超过1.6%。她的疾病似乎是肠淋巴管扩张症的典型病例。她接受了13年的对症治疗，病情基本没有变化。1969年，该患者发展为IV期弥漫性、未分化（非伯基特氏）恶性淋巴瘤。通过免疫过氧化物酶染色发现，肿瘤细胞含有细胞质IgMκ，提示该淋巴瘤起源于单克隆B细胞。她接受环磷酰胺、长春新碱和泼尼松治疗后获得成功。在开始这种全身联合化疗后不久，她的血清蛋白浓度恢复正常，水肿消退，胃肠道症状也得到治愈。此外，重复检查显示她的蛋白流失已降至仅2%。联合化疗同时治愈肠淋巴管扩张症和淋巴瘤，提示了这些病症之间的新关系，以及治疗与严重胃肠道蛋白流失相关的后天性肠淋巴管扩张症的新可能性。
