Primary intestinal lymphangiectasia is a rare disorder that may result in protein-losing enteropathy. We report a 21-year-old man with malabsorption syndrome, an unintentional weight loss of 30 kg over 10 months, lymphocytopenia, and hepatic aminotransferase elevation. His diagnosis was established by a combination of enteroscopy, histopathology, and secondary etiology exclusion. Institution of parenteral nutrition, followed by a low-fat diet, medium-chain triglycerides, and octreotide, resulted in the resolution of his symptoms and laboratory abnormalities and led to weight gain. Aminotransferase abnormalities are an atypical finding in primary intestinal lymphangiectasia and were most likely due to nonalcohol steatohepatitis after rapid weight loss. Primary intestinal lymphangiectasia should be considered in patients with protein-losing enteropathy and lymphocytopenia.
© 2022 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of The American College of Gastroenterology.