Biermann J, Meinshausen J, Kuhlmann U
Innere Abteilung 4, Robert-Bosch-Krankenhaus Stuttgart.
Dtsch Med Wochenschr. 1991 Sep 27;116(39):1473-6. doi: 10.1055/s-2008-1063775.
A 20-year-old man with severe physical weakness (from which he soon recovered spontaneously) was found to have hypoproteinaemia and lymphocytopenia (768/microliters). Ten years later, after having been free of symptoms in the meantime, oedema, ascites and abdominal pain occurred, associated with loss of physical capacity. Enteric loss of protein (alpha 1-antitrypsin clearance increased about thirtyfold) with a total serum protein concentration of only 3.7 g/dl, as well as histological evidence of lymphangiectasia of the small and large intestines provided the diagnosis of primary intestinal lymphangiectasia. In the course of the disease cholelithiasis and (after treatment with tranexamic acid) thrombosis of the axillary vein occurred. No treatment has been of any avail and the patient has been unable to work for three years.
一名20岁男性，有严重身体虚弱症状（很快自行恢复），发现有低蛋白血症和淋巴细胞减少（768/微升）。十年后，在此期间无症状，之后出现水肿、腹水和腹痛，并伴有体力下降。肠道蛋白丢失（α1-抗胰蛋白酶清除率增加约30倍），血清总蛋白浓度仅为3.7 g/dl，以及小肠和大肠淋巴管扩张的组织学证据，确诊为原发性肠淋巴管扩张症。病程中发生了胆石症和（用氨甲环酸治疗后）腋静脉血栓形成。任何治疗均无效，患者已无法工作三年。
