Primary Intestinal Lymphangiectasia (PIL) is a rare gastroenterological disorder characterized by dilated Lymph vessels in the mucosal and submucosal layers of the small intestine, which can cause protein-losing enteropathy. It can present with diarrhea, ascites and pleural effusion besides lymphopenia, hypoalbuminemia and hypo gammaglobulinemia. Although PIL is predominantly diagnosed in children under the age of three, rare cases of Adult with new diagnosed PIL have been reported, posing diagnostic challenges due to its similarity to other conditions. We report a 36-year-old woman who presented with refractory anemia, dyspnea, lower limb edema, and lymphocytopenia. The diagnosis of PIL was ultimately confirmed through histological analysis of biopsy samples obtained via enteroscopy. Her anemia was not resolved with dietary modification and pharmacologic interventions including iron supplement, octreotide and tranexamic acid. Eventually, therapeutic trial of sirolimus successfully brought her anemia under control.

This case diagnosed by dpush entroscopy & biopsies confirmed the diagnosis

severe edema, large nodules, petechia and numerous whitish spots (snowflake-like appearance) in the duodenum and more severely in the jejunum. During the biopsy of the nodules A pink fluid was extracted from the biopsy site, Which raised the suspicion about lymph component and lymphangiectasia