Waisberg Jaques, Anderi Amanda do Val, Cardoso Pedro Augusto Soffner, Borducchi José Henrique Miranda, Germini Demetrius Eduardo, Franco Maria Isete Fares, Vasconcellos Cidia
Department of Surgery, Hospital do Servidor Público Estadual de São Paulo, Avenida Ibirapuera 981, 2o andar - Vila Clementino, Sao Paulo, SP, 04029-000, Brazil.
Department of Pathology, Hospital do Servidor Público Estadual de São Paulo, Sao Paulo, SP, 04029-000, Brazil.
J Med Case Rep. 2017 Jul 13;11(1):190. doi: 10.1186/s13256-017-1340-1.
Multiple lymphomatous polyposis is a rare type of gastrointestinal lymphoma that extensively infiltrates the intestine. Multiple lymphomatous polyposis originates from the mantle zone of the lymphoma follicle and is considered to be a mantle cell lymphoma, which is a relatively aggressive type of B-cell non-Hodgkin's lymphoma. We report an unusual case of a patient with multiple lymphomatous polyposis with extensive colorectal involvement and acute intestinal obstruction, an atypical complication of this rare disease. On the basis of this case study, the pitfalls in gastrointestinal tract lymphomatous polyposis diagnosis and prognosis, as well as the treatment options, are discussed.
Our patient was a 76-year-old white woman with asthenia, cramps, and swelling in the lower left quadrant of the abdomen, as well as weight loss within the previous 5 months. A colonoscopy revealed polyps in the rectum, sigmoid colon, descending colon, and right and left colic flexures. A biopsy revealed lymphomatous infiltration of the intestinal wall. Because of the large size of the polypoid masses, which narrowed the colonic lumen in multiple locations, the patient developed acute intestinal obstruction and was referred for laparotomy. She underwent a total proctocolectomy with a permanent ileostomy and a left salpingo-oophorectomy. Microscopic examination showed the presence of a multicentric, low-grade, small lymphocytic lymphoma. Immunohistochemical analysis revealed positive immunostaining for CD79a, CD20, and CD45. These results were consistent with the diagnosis of mantle cell lymphoma. Two weeks after surgery and prior to discharge, but before the beginning of chemotherapy, the patient's general condition worsened as she experienced a severe and progressive respiratory tract infection, advanced respiratory insufficiency, and septic shock, and she ultimately died.
Mantle cell lymphoma develops as a progressive and aggressive disease with widespread polyposis of the gastrointestinal tract. The intensive chemotherapeutic regimens usually result in the regression of macroscopic and microscopic lesions; however, remissions are short in duration, and the median length of patient survival is 3-4 years. Mantle cell lymphoma is a rare disease that should be part of the differential diagnosis of polypoid diseases of the large intestine.
多发性淋巴瘤性息肉病是一种罕见的胃肠道淋巴瘤，广泛浸润肠道。多发性淋巴瘤性息肉病起源于淋巴瘤滤泡的套区，被认为是套细胞淋巴瘤，这是一种相对侵袭性的B细胞非霍奇金淋巴瘤。我们报告了一例不寻常的多发性淋巴瘤性息肉病患者，其结直肠广泛受累并出现急性肠梗阻，这是这种罕见疾病的一种非典型并发症。基于该病例研究，讨论了胃肠道淋巴瘤性息肉病诊断和预后的陷阱以及治疗选择。
我们的患者是一名76岁的白人女性，有乏力、腹痛和左下腹肿胀症状，且在过去5个月内体重减轻。结肠镜检查发现直肠、乙状结肠、降结肠以及左右结肠弯曲处有息肉。活检显示肠壁有淋巴瘤浸润。由于息肉样肿物体积较大，多处使结肠腔狭窄，患者出现急性肠梗阻，遂转诊进行剖腹手术。她接受了全直肠结肠切除术、永久性回肠造口术和左侧输卵管卵巢切除术。显微镜检查显示存在多中心、低级别、小淋巴细胞淋巴瘤。免疫组化分析显示CD79a、CD20和CD45免疫染色呈阳性。这些结果与套细胞淋巴瘤的诊断一致。术后两周，在出院前且化疗开始前，患者的一般状况恶化，出现严重且进行性加重的呼吸道感染、严重呼吸功能不全和感染性休克，最终死亡。
套细胞淋巴瘤是一种进展性侵袭性疾病，伴有广泛的胃肠道息肉病。强化化疗方案通常会使宏观和微观病变消退；然而，缓解期较短，患者的中位生存期为3至4年。套细胞淋巴瘤是一种罕见疾病，应作为大肠息肉样疾病鉴别诊断的一部分。
