Desai Rahil, Khazey Katherine, Sandhu Hasnoor, Makar Peter, Randhawa Navkiran, Khalyfa Ahamed, Khan Mahnoor, Yarbrough Alex, Spyratos Tilemahos
Franciscan Health Olympia Fields Hospital, Olympia Fields, IL, USA.
Department of Gastroenterology, Medical College of Georgia, Augusta University, Augusta, GA, USA.
Case Rep Gastroenterol. 2024 Jun 13;18(1):313-317. doi: 10.1159/000539288. eCollection 2024 Jan-Dec.
Mantle cell lymphoma (MCL), a rare non-Hodgkin's lymphoma, exhibits a genetic translocation causing CCND1 gene overexpression, affecting 5% of NHL cases, predominantly in males aged 60-70. Typically diagnosed with advanced symptoms, MCL involves widespread disease and organ spread, being aggressive and incurable with a 1.8-9.4-year average survival. Optimal treatment depends on disease aggressiveness and age. Multiple lymphomatous polyposis (MLP), a rare MCL subtype in the GI tract, is usually present with GI symptoms.
A 71-year-old woman was diagnosed with asymptomatic MLP during MCL staging who underwent thoracentesis due to large right pleural effusion and significant axillary lymphadenopathy and was treated with a chemotherapy regimen of rituximab/cytarabine and later transitioned to bendamustine/rituximab. This patient eventually underwent a bone marrow biopsy and later a bone marrow transplant.
We present a unique case of asymptomatic MLP, emphasizing the importance of early detection for the poor prognosis of MLP with a mean survival of less than 3 years.
套细胞淋巴瘤（MCL）是一种罕见的非霍奇金淋巴瘤，表现为导致CCND1基因过表达的基因易位，占非霍奇金淋巴瘤病例的5%，主要发生在60 - 70岁的男性中。MCL通常在出现晚期症状时被诊断出来，涉及广泛的疾病和器官扩散，具有侵袭性且无法治愈，平均生存期为1.8 - 9.4年。最佳治疗方案取决于疾病的侵袭性和年龄。多发性淋巴瘤性息肉病（MLP）是胃肠道中一种罕见的MCL亚型，通常伴有胃肠道症状。
一名71岁女性在MCL分期时被诊断为无症状性MLP，因右侧大量胸腔积液和明显的腋窝淋巴结肿大接受了胸腔穿刺，并接受了利妥昔单抗/阿糖胞苷化疗方案治疗，后来转为苯达莫司汀/利妥昔单抗治疗。该患者最终接受了骨髓活检，随后进行了骨髓移植。
我们报告了一例无症状性MLP的独特病例，强调了早期检测对于MLP预后不良（平均生存期不到3年）的重要性。
