Chylous Ascites: A Rare Initial Presentation of High‐Grade Follicular Lymphoma

Background Chylous ascites is an uncommon condition characterized by the accumulation of triglyceride‐rich, milky fluid in the peritoneal cavity due to lymphatic disruption or obstruction. Although lymphoma is a leading cause of malignant chylous ascites, its occurrence as an initial presentation remains rare. Case Presentation A 69‐year‐old woman presented with a three‐month history of postprandial abdominal pain, weight loss, anorexia, and dyspnea. Imaging revealed extensive abdominal and pelvic lymphadenopathy with bilateral pleural effusions. Diagnostic laparoscopy demonstrated milky peritoneal fluid, and fluid analysis confirmed chylous ascites (triglycerides, 1361 mg/dL). Lymph node biopsy demonstrated high‐grade B‐cell lymphoma with morphological features favoring follicular lymphoma. Immunohistochemistry revealed a markedly elevated Ki‐67 proliferative index (> 90%), and genomic profiling identified pathogenic EZH2 and TET2 mutations with a high tumor mutational burden. According to the fifth edition of the WHO Classification of Haematolymphoid Tumours (2022), these findings are most consistent with follicular lymphoma, a mature B‐cell neoplasm with high‐grade features. Given the high‐output drainage and recent surgery, cytotoxic chemotherapy was deferred, and rituximab monotherapy was initiated. Rapid clinical improvement and decreased drain output allowed safe transition to standard R‐CHOP therapy, achieving a complete metabolic response (Deauville score 2) after six cycles. Conclusions This case highlights chylous ascites as a rare but important presenting feature of lymphoma. Its recognition should prompt early histopathologic evaluation and multidisciplinary management. In selected postoperative or frail patients, rituximab monotherapy can serve as an effective bridge to full chemotherapy, facilitating recovery and improving outcomes. Early diagnosis and targeted treatment remain essential to prevent complications from lymphatic loss and to optimize prognosis in lymphoma‐associated chylous ascites.