无胃肠道症状的多发性淋巴瘤性息肉病:套细胞淋巴瘤的非典型病例。

IF 0.5 Q4 GASTROENTEROLOGY & HEPATOLOGY Case Reports in Gastroenterology Pub Date : 2024-06-13 eCollection Date: 2024-01-01 DOI:10.1159/000539288
Rahil Desai, Katherine Khazey, Hasnoor Sandhu, Peter Makar, Navkiran Randhawa, Ahamed Khalyfa, Mahnoor Khan, Alex Yarbrough, Tilemahos Spyratos
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引用次数: 0

摘要

简介:套细胞淋巴瘤(MCL)是一种罕见的非霍奇金淋巴瘤:套细胞淋巴瘤(MCL)是一种罕见的非霍奇金淋巴瘤,其基因易位导致CCND1基因过度表达,占NHL病例的5%,主要发生于60-70岁的男性。MCL 通常在确诊时已出现晚期症状,病变范围广,可向器官扩散,具有侵袭性,无法治愈,平均生存期为 1.8-9.4 年。最佳治疗方法取决于疾病的侵袭性和年龄。多发性淋巴瘤性息肉病(MLP)是一种罕见的消化道 MCL 亚型,通常伴有消化道症状:一名71岁的妇女在MCL分期时被诊断为无症状MLP,因右侧胸腔大量积液和明显的腋窝淋巴结病而接受了胸腔穿刺术,并接受了利妥昔单抗/阿糖胞苷的化疗方案,后来又过渡到苯达莫司汀/利妥昔单抗。该患者最终接受了骨髓活检,随后进行了骨髓移植:我们介绍了一例独特的无症状MLP病例,强调了早期发现的重要性,因为MLP预后较差,平均存活期不到3年。
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Gastrointestinal Symptom-Free Multiple Lymphomatous Polyposis: An Atypical Case Presentation of Mantle Cell Lymphoma.

Introduction: 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.

Case presentation: 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.

Conclusion: 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.

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来源期刊
Case Reports in Gastroenterology
Case Reports in Gastroenterology Medicine-Gastroenterology
CiteScore
1.10
自引率
0.00%
发文量
99
审稿时长
7 weeks
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