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Query: UMLS:C0079731 (B-cell lymphoma)
 16,671 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Hereditary non-polyposis colon cancer (HNPCC) is an autosomal dominant disorder featuring familial clustering of colorectal and/or endometrial cancer, and other malignancies. Except for a rare case report, Hodgkin's disease (HD) and non-Hodgkin's lymphoma (NHL) have not been considered part of HNPCC. Recent murine models for HNPCC have shown an increased incidence of B- and T-cell lymphoma, as well as tumors of the gastrointestinal tract and other organ systems, involving defects in genes resulting in faulty mismatch repair (MMR) of DNA. These MMR genes include MLH1, MSH2, MSH3, MSH6, PMS1 and PMS2. We sought to analyze the occurrence of NHL and HD in families with clusters of colorectal cancers (CRC). Probands from 21 kindreds were classified as HNPCC (3), HNPCC-like (5), and HNPCC-variant (13); seen and followed by Clinical Genetics at Memorial Hospital the kindreds were assessed for the occurrence of NHL or HD. Of the 21 pedigrees, a total of 37 patients were identified who were diagnosed with leukemia, lymphoma, or HD. Fourteen of the 37 patients with a diagnosis of NHL or HD were further classified and showed varying histologies ranging from chronic lymphocytic leukemia/small lymphocytic lymphoma (2), mycosis fungoides (1), follicular lymphoma (1), extranodal marginal zone lymphoma of MALT type (2), diffuse large B-cell lymphoma (4), nodular sclerosis HD (3), and mixed cellularity HD (1). Microsatellite instability studies were performed on 6 cases but none showed evidence of replication error repair defects. Immunohistochemical stains performed on paraffin sections from these 6 representative cases showed differential protein expression of MLH1, MSH2, MSH6, and PMS2 when compared to normal reactive tissues from the same patient but showed no significant differences when compared to controls of non-familial, sporadic lymphomas. These results suggest that lymphomas arising in the setting of familial CRC do not bear the molecular hallmarks of HNPCC. Further studies are needed to explain the differential patterns of expression of RER-associated proteins in lymphomas, as well as the association of lymphomas and possibly renal cell cancers in a subset of kindreds in which CRC clustering is evident.
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PMID:Analysis of mismatch repair defects in the familial occurrence of lymphoma and colorectal cancer. 1240 Jun 5

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.
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PMID:A rare case of multiple lymphomatous polyposis with widespread involvement of the gastrointestinal tract. 1287 80

A 50-year old woman suffering from diabetes had a CT scan that revealed a diffuse thickening of small intestinal wall and swollen paraaortic lymph nodes. An esophagogastroduodenoscopy (EGD) confirmed multiple polypoid lesions in the duodenum and small intestine, and conventional histological testing revealed non-specific inflammatory changes. Further examinations including the immunohistochemical profiles of the biopsied specimens led us to diagnose the lesion as a marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue type, forming multiple lymphomatous polyposis sequentially spreading from duodenal bulb to terminal ileum. According to Lugano's classification, its staging was clinically diagnosed as stage II. Two courses of a standard CHOP (cyclophosphamide, doxorubicin hydrochloride, vincristine sulfate, and predonisolone) regimen with rituximab reduced the lesion and the patient had a almost complete response. A 5-year follow-up EGD and histological examinations detected no recurrence of the disease.
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PMID:A case of mucosa-associated lymphoid tissue lymphoma forming multiple lymphomatous polyposis in the small intestine. 1745 82

The clinicopathologic features of malignant lymphoma of the small intestine were reviewed. Genetically, characteristic chromosomal translocations have been identified in several B-cell lymphomas, such as t (11 ; 18)/API2-MALT1 in MALT lymphoma, t (14 ; 18)/IGH-BCL2 in follicular lymphoma, or t (3 ; 14)/BCL6-IGH in diffuse large B-cell lymphoma (DLBCL). Histologically, DLBCL is most frequently observed, and T-cell lymphoma and follicular lymphoma are more frequent in small intestinal cases than in gastric cases. Macroscopically, small intestinal lymphomas are classified as polypoid, ulcerative (including stricturing, non-stricturing and aneurysmal forms on radiography), multiple lymphomatous polyposis, diffuse, or other types. A significant correlation is observed between these macroscopic/radiographic and histologic types. The therapeutic strategy, such as surgery, chemotherapy, antibiotics or watch-and-wait, should be determined based on the disease extent, histologic type, and clinical stage.
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PMID:[Malignant lymphoma of the small intestine]. 1861 20

Primary gastrointestinal (GI) lymphomas constitute 5%-10% of all gastrointestinal tumors. Involvement of the entire GI tract as multiple polypoid lesions is very rare. Multiple lymphomatous polyposis is a distinctive and rare type of malignant GI lymphoma. Multiple lymphomatous polyposis is thought to represent mantle cell lymphoma of the GI tract. Approximately 60 cases of MLP have been reported so far. We report the first case of diffuse large B-cell lymphoma presenting as multiple lymphomatous polyposis of the GI tract in a 49-year-old patient with HIV and describe the clinicopathologic features. The patient was treated with a combination therapy of highly active antiretroviral therapy and CHOP (cyclophosphamide/doxorubicin/vincristine/prednisone) and exhibited complete remission.
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PMID:Diffuse large B-cell lymphoma presenting as multiple lymphomatous polyposis of the gastrointestinal tract. 1865 Jan 83

Multiple lymphomatous polyposis (MLP) is a rare extra-nodal manifestation of lymphoma. In most cases, MLP is associated with mantle cell lymphoma (MCL). We report a 66-year-old male diagnosed with small lymphocytic lymphoma (SLL)/chronic lymphocytic lymphoma (CLL), who showed evidence of rectal bleeding. A CT-scan of the abdomen and pelvis showed an enlarged spleen, multiple paraaortic and mesenteric lymph nodes, and some diverticular pouching along the antimesenteric border of the pelvic colon. A colonoscopy revealed the presence of multiple polypoid lesions, biopsies of which showed diffuse lymphoid infiltrate without any identifiable follicles. Immunohistochemical analysis combined with a Fluorescence In-Situ Hybridization (FISH) study excluded the diagnosis of MCL. A bone marrow aspiration biopsy demonstrated diffuse infiltration of the bone marrow with low grade lymphocytes that expressed CD 20, CD5 and CD23, with negative BCL-1, t (11; 14) and cyclin D1. A diagnosis of B-cell CLL with kappa light chain restriction was made. Multiple lymphomatous polyposis is considered to be a digestive counterpart to MCL and can therefore be considered as a presentation of MCL. In our case, the polypoid lesions failed to show the characteristic features of MCL. The patient's bone marrow revealed a B-cell lymphoma of CLL/SLL phenotype, which to our knowledge has not been linked to MLP in previously reported cases.
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PMID:Multiple lymphomatous polyposis associated with small lymphocytic lymphoma: a unique presentation. 1910 11

Mantle cell lymphoma (MCL) is an aggressive B cell lymphoma with frequent involvement of the gastrointestinal tract and peripheral blood (PB). In addition to the B cell markers, the neoplastic cells express CD5 and CD43. In patients with a prior history of MCL with PB involvement, the appearance of leukemic cells after chemotherapy usually heralds a relapse, particularly if the leukemic cells express B cell markers and CD43. We report a patient with MCL who presented with multiple lymphomatous polyposis of the intestine. The staging procedures revealed the involvement of lymph nodes, bone marrow and PB. Three years after chemotherapy, thrombocytopenia with the appearance of rare leukemic cells in the PB was noted. Leukemic cells obtained from bone marrow aspirate expressed CD19 and CD43, suggesting a relapse. Detailed cytomorphological and immunophenotypic studies unveiled the myeloid nature of these leukemic cells, and a diagnosis of therapy-related acute myeloid leukemia was made. This case illustrates the importance of morphologic examination and performing a complete antibody panel in the diagnosis of a suspected relapse in patients with a prior history of lymphoma.
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PMID:Aberrant expression of CD19 and CD43 in a patient with therapy-related acute myeloid leukemia and a history of mantle cell lymphoma. 1960 31

Lymphomas of the gall bladder and extrahepatic bile ducts are exceedingly rare. We present the clinicopathological features of 19 cases from our files; 14 patients had primary lymphoma (13 involving gall bladder and 1 involving common hepatic duct), while 5 had systemic lymphoma on further work-up. Most patients presented with symptoms mimicking cholecystitis. The most common primary lymphoma types were diffuse large B-cell lymphoma, extranodal marginal zone lymphoma, B-lymphoblastic lymphoma, and follicular lymphoma. Two cases had features of lymphomatous polyposis, one a case of follicular lymphoma and the second a case of mantle cell lymphoma, with disease limited to the mantle zones, so-called in situ mantle cell lymphoma. Other rare lymphoma subtypes not described earlier in this site included the extracavitary variant of primary effusion lymphoma and plasmablastic lymphoma. Patients with diffuse large B-cell lymphoma and extranodal marginal zone lymphoma were older (mean age 75.8 y) than those with other subtypes (mean age 47 y) and more likely to have gallstones (60% vs. 12.5%). A comprehensive literature review revealed 36 primary gall bladder and 16 primary extrahepatic bile duct lymphomas. When compared with primary gall bladder lymphomas, those involving the extrahepatic bile ducts present at a younger age (47 y vs. 63 y) usually with obstructive jaundice, and are less often associated with gallstones (17% vs. 50%) or regional lymph node involvement (6% vs. 31%). In conclusion, primary lymphomas of the gall bladder and extrahepatic bile ducts show a broad spectrum of disease types, but in many respects mirror the spectrum of primary lymphomas of the gastrointestinal tract.
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PMID:Gall bladder and extrahepatic bile duct lymphomas: clinicopathological observations and biological implications. 2067 81

Primary mantle cell lymphoma of the digestive tract is uncommon; it rarely involves the stomach and generally manifests as a lymphomatous polyposis. Isolated gastric localization is exceedingly rare. We present a case of a 57-year-old man who presented with a six-month history of atypical epigastric pain. Endoscopy revealed a large ulceration of the gastric mucosa. Histological examination of the biopsied specimens concluded to a marginal zone B-cell lymphoma (MALT-type) of low-grade. Patient underwent two courses of anti-Helicobacter pylori therapy followed by chemotherapy, without endoscopic amelioration. Histological control showed the persistence of a nodular lymphomatous proliferation that respects glands. It was made of monotonous small cells reactive with CD5 and cyclin D1. The diagnosis of gastric mantle cell lymphoma was made. No other digestive or nodal localization was found. Patient underwent intensive chemotherapy anti-CD20; he was free of disease three years after diagnosis. Primary mantle cell lymphoma of the stomach is an uncommon neoplasm. Diagnosis is often difficult and requires the use of CD5 to exclude a MALT-cell lymphoma, which is much more frequent in this location.
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PMID:[Isolated primary gastric localization of a mantle cell lymphoma]. 2083 46

Multiple lymphomatous polyposis is an uncommon type of primary non-Hodgkin's gastrointestinal B cell lymphoma. It is considered to represent mantle cell lymphoma of the gastrointestinal tract mainly. However, rarely some cases of multiple lymphomatous polyposis belong to follicular lymphoma, small lymphocytic lymphoma, diffuse large cell lymphoma, or mucosa-associated lymphoid tissue lymphomas. Recently, WHO 2008 classification established a new diagnostic category of B cell lymphoma, unclassifiable, with features intermediate between DLBCL and BL (DLBCL/BL). To the best of our knowledge, this subcategory of lymphoma presenting as lymphomatous polyposis has never been described. We herein describe a case of lymphomatous polyposis involving entire gastrointestinal tract, which turned out as DLBCL/BL on immunohistochemistry and molecular analysis. The patient was treated with hyper-CVAD chemotherapy regimen, resulting in a complete response.
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PMID:B cell lymphoma unclassifiable with features intermediate between diffuse large B cell and burkitt lymphoma--presented with multiple lymphomatous polyposis of gastrointestinal tract. 2121 68


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