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          Multiple Lymphomatous Polyposis 2

                           

 

A rare case of multiple lymphomatous polyposis with widespread involvement of the gastrointestinal tract. Arch Pathol Lab Med. 2003 Aug;127(8):1028-30.

Multiple lymphomatous polyposis (MLP) is an uncommon type of primary non-Hodgkin gastrointestinal (GI) B-cell lymphoma characterized by the presence of multiple polyps along the GI tract. Malignant cells of MLP have mantle cell characteristics and thus are considered to be the counterpart of the mantle cell lymphoma (MCL) in the GI tract. Since 1961, no more than 70 well-documented cases have been published. We report the case of 53-year-old man diagnosed as having MLP. The patient presented with diffuse abdominal pain, chronic lower GI bleeding, peripheral lymphadenopathy, and weight loss. The lymphomatous polyps extended from the esophagus to the rectum, with bone marrow infiltration. Immunohistologic findings were characteristic of MCL. The patient was treated with a combined cyclophosphamide, vincristine, and prednisone chemotherapy regimen, resulting in a partial response.

Diffuse lymphomatous polyposis of the gastrointestinal tract. A case report with immunohistochemical studies.Gastroenterology. 1985 May;88(5 Pt 1):1267-70.

A case of diffuse lymphomatous polyposis of the gastrointestinal tract is reported. The patient presented with abdominal pain and weight loss and x-rays revealed multiple polyps involving the entire gastrointestinal tract. Biopsies confirmed the diagnosis of lymphomatous polyposis. The patient also had involvement of the bone marrow and supraclavicular lymph node. Immunologically this lymphoma was characterized as a monoclonal proliferation of B lymphocytes bearing immunoglobulin M, type kappa. Diffuse lymphomatous polyposis of the gastrointestinal tract appears to be a generalized malignancy of uncommitted B cells of Peyer's patches. The migratory properties of these cells may account for the tendency to dissemination of lymphomatous polyposis. Diffuse lymphomatous polyposis of the gastrointestinal tract is a distinct entity, separate from the diffuse gastrointestinal lymphoma known as Mediterranean-type lymphoma.

Pathology of the Intestinal Polyp (Multiple Lymphomatous Polyposis)

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Lymphomatous polyposis of the gastrointestinal tract, including mantle cell lymphoma, follicular lymphoma and mucosa-associated lymphoid tissue lymphoma.Histopathology. 2005 Nov;47(5):467-78.

AIMS: Lymphomatous polyposis (LP) is considered to represent mantle cell lymphoma (MCL) of the gastrointestinal (GI) tract. However, a few reports have suggested that some are follicular lymphoma (FL) or mucosa-associated lymphoid tissue (MALT) lymphomas. In this study, we analysed 35 patients and clarified the clinicopathological features of LP. METHODS AND RESULTS: Paraffin-embedded tissue samples were stained immunohistochemically and analysed by tissue-fluorescence in situ hybridization (T-FISH) for IGH/CCND1 (cyclin D1) and IGH/BCL2. The average age of the patients was 58.3 years. Over half of the cases showed gastric, duodenal, small intestinal, ileocaecal and sigmoid colonic lesions (15, 19, 15, 16 and 16 cases, respectively). Phenotypically, cases were classified into three types of MCL (cyclin D1+ CD5+ CD10-) (n=12), FL (cyclin D1- CD5- CD10+) (n=14) and MALT (cyclin D1- CD5- CD10-) (n=9). T-FISH identified 11 of the 11 examined cases with MCLs to have IGH/CCND1, while seven of 10 cases with FL had IGH/BCL2, and none of the MALT cases were positive for IGH/CCND1 or IGH/BCL2. At the study endpoint, five of 12 patients with MCL were dead, two of 14 with FL and one of nine with MALT were dead of other disease. Event-free survival analysis showed significantly poorest outcome in MCL, followed by FL, while MALT was associated with a favourable outcome (P=0.0040). CONCLUSIONS: Our study emphasizes the importance of differentiating MCL, FL and MALT of LP in evaluating prognosis and hence the most suitable therapeutic regimen.

 
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