伪装成盲肠肿块的套细胞淋巴瘤一例。

IF 0.6 Q4 GASTROENTEROLOGY & HEPATOLOGY
Case Reports in Gastrointestinal Medicine Pub Date : 2021-09-10 eCollection Date: 2021-01-01 DOI:10.1155/2021/5581043
Sarahi Herrera-Gonzalez, Dema Shamoon, Tingliang Shen, Simon Badin, Yatinder Bains
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引用次数: 0

摘要

套细胞淋巴瘤(MCL)是一种b细胞非霍奇金淋巴瘤,是一种罕见的侵袭性疾病,由于其诊断时的表现较晚,预后较差。其特点是Bcl-1基因易位,导致细胞周期蛋白D1过表达。MCL常以多发性淋巴瘤性息肉病(MLP)的形式出现,其中在胃肠道(GI)中观察到无数息肉。在极少数情况下,MCL表现为单个肿块。最常见的表现为60多岁的男性患者,伴有全身性淋巴结病变、淋巴结外受累和B型症状(盗汗、发烧和体重减轻)。MLP的内镜表现包括胃粘膜的脑样折叠和从十二指肠延伸到大肠的无数息肉,约占所有胃肠道淋巴瘤的9%。不太常见的是,只有2-4%的胃肠道恶性肿瘤表现为原发性胃肠道MCL为单个肿块,通常发生在胃和肠的回盲区。影像学表现包括淋巴结病、脾肿大、多发性息肉病、壁增厚伴溃疡或肿块形成。在大多数情况下,在诊断时发现晚期疾病,即使开始适当治疗,其5年生存率仅为26%至46%。高有丝分裂率或Ki-67指数具有预后价值,与预后不良有关。治疗包括常规的化学免疫治疗,包括R CHOP(利妥昔单抗、环磷酰胺、阿霉素、长春新碱和强尼松)或RB(利妥昔单抗和苯达莫司汀),后者耐受性更好,无进展生存期更长。手术切除通常限于出现出血、穿孔或肠梗阻等并发症的患者。我们提出一个独特的情况下,70岁的男性与非胆汁,非血性呕吐,并有症状性贫血谁被发现有盲肠团一致的MCL。
本文章由计算机程序翻译,如有差异,请以英文原文为准。

A Unique Case of Mantle Cell Lymphoma Masquerading as a Cecal Mass.

A Unique Case of Mantle Cell Lymphoma Masquerading as a Cecal Mass.

A Unique Case of Mantle Cell Lymphoma Masquerading as a Cecal Mass.

A Unique Case of Mantle Cell Lymphoma Masquerading as a Cecal Mass.

Mantle cell lymphoma (MCL), a type of B-cell non-Hodgkin's lymphoma, is a rare and aggressive disease with a poor prognosis due to its advanced presentation at diagnosis. It is characterized by a translocation in the Bcl-1 gene, which results in overexpression of cyclin D1. MCL is frequently seen in the form of multiple lymphomatous polyposis (MLP) in which innumerable polyps are observed in the gastrointestinal (GI) tract. In rare instances, MCL presents a single mass. The most common presentation involves male patients in their sixties, with generalized lymphadenopathy, extranodal involvement, and B symptoms (night sweats, fever, and weight loss). Endoscopic findings of MLP include cerebroid folding of the gastric mucosa and innumerable polyps extending from the duodenum to the large intestine and are reported in approximately 9% of all GI lymphomas. Less commonly, only 2-4% of GI malignancies present as a primary GI MCL as a single mass, usually in the stomach and ileocecal region in the intestine. Radiologic findings include lymphadenopathy, splenomegaly, multiple polyposis, or wall thickening with ulceration or mass formation. In most instances, advanced disease is found at diagnosis, for which 5-year survival ranges only from 26 to 46%, even when appropriate treatment is initiated. High mitotic rate, or Ki-67 index, is of prognostic value and is associated with poor prognosis. Treatment involves conventional chemo-immunotherapy consisting of R CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone) or RB (rituximab and bendamustine), with the latter being better tolerated and associated with longer progression-free survival. Surgical resection is usually limited to patients in which complications are seen such as bleeding, perforation, or bowel obstruction. We present a unique case of a 70-year-old male with nonbilious, nonbloody emesis, and symptomatic anemia who was found to have a cecal mass consistent with MCL.

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Case Reports in Gastrointestinal Medicine
Case Reports in Gastrointestinal Medicine GASTROENTEROLOGY & HEPATOLOGY-
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