Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019829 (Hodgkin's disease)
 30,247 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Primary gastrointestinal lymphoma is a rare condition. It constitutes approximately 5% of all lymphoid malignancies. Primary lymphomas of the intestinal tract rarely present as multiple polyposis. We describe such a patient who was treated with chemotherapy. The treatment of choice is surgery when possible. Chemotherapy is used in addition when high grade non-Hodgkin lymphoma is revealed. Chemotherapy should be used for patients who are unable to undergo curative resections. Prognosis is poor for patients with a non-resectable disease.
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PMID:[Intestinal lymphoma presented as multiple intestinal polyposis]. 150 56

Two years after endoscopic excision of several polyps of the colon (histological examination revealing infiltration by a malignant non-Hodgkin lymphoma) a 67-year-old man was again found to have blood and mucus in his stool. Because local recurrence was suspected a coloscopy was performed. This revealed multiple polyps throughout the entire colon, predominantly in the sigmoid and rectum. Biopsy showed focal infiltration of the submucosa and mucosa with centrocytic non-Hodgkin lymphoma, compatible with the diagnosis of malignant lymphomatous polyposis of the gastrointestinal tract. Gastroduodenoscopy then demonstrated duodenal polyps and massive coarsening of the gastric folds due to infiltration. For the first time tumour masses were also found in the epipharynx. After 6 chemotherapy cycles according to the CHOP scheme (day 1: cyclophosphamide, 1500 mg, adriblastin, 100 mg and vincristine, 2 mg; days 1-5: prednisolone, 100 mg) all lymphoma infiltrates regressed completely. The patient has been symptom-free for 9 months.
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PMID:[Malignant lymphomatous polyposis of the gastrointestinal tract]. 173 Jan 94

Only a small proportion of cancers, arising in inherited syndromes such as polyposis coli, have an unequivocally inherited basis. Nevertheless, most common cancers show familial clustering, much of which may be due to inherited predisposition. If so, there may be wide variation in genetic susceptibility to common cancers. The precise models of susceptibility are unclear, but for ovarian cancer and breast cancer there is some evidence that a small proportion of cases result from highly penetrant dominant genes. This has been confirmed recently for breast cancer by genetic linkage studies. Clear evidence for genetic susceptibility has been obtained for Hodgkin's disease and nasopharyngeal carcinoma, where the existence of susceptibility genes at the HLA locus has been demonstrated by linkage analysis. These genes could account for the majority of cases of these cancers. Identification of other cancer susceptibility genes should be possible, either directly using linkage analysis, or through identification of constitutional phenotypes related to cancer risk.
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PMID:The contribution of inherited predisposition to cancer incidence. 210 19

Multiple lymphomatous polyposis of the gastrointestinal tract was initially described as mucosal lymphomatous involvement by any of a variety of Hodgkin's or non-Hodgkin's lymphomas that produced a polypoid appearance over long segments of the gastrointestinal tract. We studied four patients in whom histology revealed diffuse small cleaved cell lymphoma (one case), or intermediate lymphocytic lymphoma of diffuse type (one case), or mantle zone pattern (two cases). All four cases are classifiable as centrocytic lymphoma. Cell suspension and immunocytochemical studies demonstrated B-cells of IgMD or M type with light chain restriction (two kappa, two lambda) showing a B1+ HLA Dr+ LN2+ CD5+ CD10+. Although all four patients had a partial response to combination chemotherapy, three of them died within 3 years. Analysis of 24 cases reported since 1971 (including the present cases) suggests that MLP is a distinct clinicopathological entity that results from gastrointestinal involvement by a B-cell centrocytic lymphoma. It is distinct from the recently described clinicopathological forms of centrocytic lymphoma and intermediate lymphocytic lymphoma, which both show extensive peripheral lymphadenopathy and splenomegaly, but it is probably closely related to them. The differences are probably attributable to distinct cell tropism or homing properties rather than to cellular histogenesis or degree of maturation.
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PMID:Multiple lymphomatous polyposis of the gastrointestinal tract. A clinicopathologically distinctive form of non-Hodgkin's lymphoma of B-cell centrocytic type. 266 36

Two cancer-prone families are reported. In the first one four first-degree relatives over three generations presented a colonic carcinoma, three of them at a proximal anatomic site. For grandmother and father these occurred at ages of 43 and 54 years, respectively, for the son and the daughter at ages of 26 and 22. The grandmother underwent a palliative ileotransversostomy, surgery typically associated with a bad prognosis, but she survived for forty years that initial neoplasm and had an hysterectomy with oophorectomy at age of 63 for endometrial malignancy; she deceased at age of 83 a few days after surgical treatment of tumoral small bowel obstruction: pathological evaluation disclosed a fourth cancer on first duodenum. The second kindred shows over three generations 11 cancer-affected individuals, three of them with double primary cancer: breast and sigmoid, breast and endometrium, colon and Hodgkin disease. This pedigree includes 8 colorectal neoplasms occurring at 47 years of mean age. These findings are consistent with the cancer-family syndrome and hereditary non-polyposis colon cancer described by Henry Lynch upon four criteria: high frequency of adenocarcinoma, excess of multiple primary malignancies, synchronous or metachronous, early age of onset of cancer and autosomal dominant inheritance. Moreover the hereditary colon cancer is usually localised to the proximal colon, not associated to polyposis coli and allows a prolonged survival. Up to day such families are only identified by pedigree data. The identification of a cancer-prone family calls for an active follow-up of relatives putatively at risk starting at the age of 15 to 20.
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PMID:[Familial cancer of the colon without polyposis and the familial cancer syndrome. Apropos of 2 cases over 3 generations]. 666 87

Malignant diseases of the lymphoreticular system can become manifest within the gastrointestinal tract, either isolated, involving solitary organs, in generalized form, or as diffuse polyposis. The ratio of primary to secondary lymphomas is quoted as being 1:2 to 1:3. The clinical picture is characterized by their spread. Abdominal pain, loss of weight, anorexia, nausea and vomiting, including even gastrointestinal hemorrhage, are the most frequently observed symptoms. The involvement of the gastrointestinal tract in Hodgkin's and non-Hodgkin lymphomas of, on average, some 10%, is confirmed in our own material over the past 7 years. From a total of 580 malignant lymphomas, 47 cases were confirmed, endoscopically and bioptically, to have gastrointestinal involvement, primary lymphomas accounting for 1/3, secondary for 2/3. Gastric lymphomas accounted for 59.6%, small-bowel lymphomas for 25.5%, a large-bowel involvement was seen in 14.9%. The centrocytic-centroblastic and lymphoblastic lymphomas predominate.
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PMID:[Gastrointestinal manifestation of malignant systemic diseases]. 668 18

Genetic instability has been recently related to point mutations on genes involved in DNA repair pathway of errors produced during replication. These molecular alterations have been described in hereditary and sporadic colon carcinomas and related tumors. To examine genetic instability on lympho- and myeloproliferative processes, we analyzed the behaviour of 10 microsatellite markers and one VNTR on different chromosomes in 10 patients with non-Hodgkin lymphomas (NHL), one patient with acute lymphoblastic leukemia (ALL) and 10 patients with acute myeloid leukemia (AML). Mobility shifts were found in three of those cases. One of them showed genetic instability for several markers--microsatellites and VNTR- and the other two showed differences for only one marker. As a correlation between point mutations in MSH2 gene and the presence of genetic instability in hereditary non-polyposis colon cancer (HNPCC) and related tumors has been found, we analyzed the sequence of a conversed region of this gene in the cases showing this phenomenon. We only found a polymorphism, previously described, in 669 codon from cDNA.
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PMID:Genetic instability of microsatellites in hematological neoplasms. 759 85

Primary gastrointestinal lymphoma comprises a group of distinctive clinicopathological entities, most of which are not included in current lymph node-based lymphoma classifications. They may be of B- or T-cell type, with primary gastrointestinal Hodgkin's disease being extremely uncommon. Most low grade B-cell gastrointestinal lymphomas are of mucosa-associated lymphoid tissue (MALT) type, so called because they recapitulate the features of MALT rather than those of lymph nodes. Paradoxically, however, most MALT lymphomas arise in the stomach, which normally contains no organized lymphoid tissue. These gastric MALT lymphomas appear to arise in MALT acquired as a reaction to infection of the stomach by Helicobacter pylori and their growth can be influenced by eradication of this organism from the stomach. Low grade MALT lymphomas, which usually have a very favorable clinical course, may undergo high grade transformation; high grade tumours also may arise de novo and these probably also belong to the MALT group. Immunoproliferative small intestinal disease (IPSID) is a special form of MALT lymphoma with a restricted geographic distribution, which is characterized by synthesis of alpha heavy-chain immunoglobulin. Other gastrointestinal B-cell lymphomas include mantle cell lymphoma, which presents as lymphomatous polyposis, and Burkitt's or Burkitt-like lymphoma. Enteropathy (celiac disease)-associated T-cell lymphoma (EATL) is the most common primary gastrointestinal T-cell lymphoma. This is a clinically aggressive tumor that arises from the intraepithelial T-cell population, which is increased in celiac disease.
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PMID:Gastrointestinal lymphoma. 792 6

Hypertrophic osteoarthropathy (HOA), well known in adults, is rarely encountered in children. The clinical features include clubbing of the fingers and toes, arthritis, and a sometimes painful ossifying periostitis of the tubular bones. Apart from a hereditary form (primary HOA), most of the cases encountered in children are secondary and associated with conditions such as chronic suppurative lung processes (e.g., cystic fibrosis), congenital heart disease, biliary atresia, and polyposis coli. The association with malignant disorders, which is relatively common in adults, is very rare in children. In 1986 the authors published a case report of a patient with carcinoma of the nasopharynx who developed HOA. Another similar patient has been encountered. In both, the appearance of HOA was associated with a very poor prognosis. A meticulous research of the literature from 1890 to 1990 revealed only 24 children (19 boys, 5 girls) under the age of 18, with malignancy and associated HOA. Among them were 10 patients with a carcinoma of the nasopharynx, 8 with osteosarcoma, 3 with Hodgkin's lymphoma, 1 with a periosteal sarcoma, 1 with mesothelioma of the pleura, and 1 with carcinoma of the thymus. In five patients with HOA, there were no abnormalities of the lungs, mediastinum, or pleura, and none developed during the course of the disease. Many authors mention the predictive value of HOA, especially in association with malignant tumors. In contrast to suppurative processes in the lungs, in those with neoplastic disease involving the chest, HOA may precede pulmonary symptoms by 1-18 months. A striking feature of HOA in these instances is the reversibility of the complaints after successful treatment of the disorder of the chest, both in benign and malignant conditions. The present case is the second reported by the authors and the first description of a girl with carcinoma of the nasopharynx developing HOA.
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PMID:Hypertrophic osteoarthropathy in childhood malignancy. 841 3

Non-Hodgkin lymphomas affecting extranodal tissues can be primitive or secondary. The area most frequently involved is the gastrointestinal tract. MALT-type lymphomas are B-cell lymphomas characterized by small cells originating from the lymphoid tissue of the mucosa. The criteria for a correct diagnosis of extranodal lymphomas are morphological, but include immunophenotypic and immunogenotypic analysis. Gastrointestinal lymphomas consist of B-cell and T-cell lymphomas. B cell-lymphomas include: small cell B-lymphomas, large cell B-lymphomas, immunoproliferative small intestinal disease; multiple lymphomatous polyposis, follicular lymphomas, B-cell lymphomas centrocytic type. T-cell lymphomas include enteropathy-associated and non enteropathy-associated lymphomas.
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PMID:[Gastrointestinal lymphomas]. 853 63


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