Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0021831 (enteropathy)
 4,403 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Ulcerative jejunitis (UJ) and enteropathy-associated T-cell lymphoma (EATL) are closely related conditions both associated with celiac disease. Benign-appearing inflammatory ulcers are seen in both, which has led to the suggestion that UJ is a manifestation of EATL. The aim of this study was to investigate this relationship using the polymerase chain reaction (PCR) to detect T-cell gene rearrangement. PCR amplification of the T-cell receptor gamma-chain gene was performed on DNA extracted from lymphoma, associated inflammatory ulcers, and intervening mucosa in six EATL cases and from ulcers and intervening mucosa of seven cases of UJ. In two of these cases, DNA from a subsequent lymphoma was also studied. The PCR products from the tumor and an ulcer from one EATL case, two ulcers from one case of UJ, and one ulcer and subsequent cutaneous lymphoma from one UJ case were sequenced. Twenty-five ulcers from twelve cases of Crohn's disease, twenty sections of normal bowel, and nine celiac biopsies were included as controls. A monoclonal T-cell population defined by a dominant band equal in size to that amplified from the lymphoma was identified in at least one ulcer from four informative EATL cases and from intervening mucosa in three. Monoclonality was demonstrated in at least one, and up to thirteen, ulcers from all seven cases of UJ, in intervening mucosa in five, and in the two subsequent lymphomas. Sequencing showed the same clone was present in the tumor and the ulcer in the EATL case, in two of three ulcers from the UJ case, and in an ulcer and subsequent cutaneous lymphoma in one UJ case. All Crohn's disease ulcers and all sections of normal bowel were polyclonal. One of nine celiac biopsies showed a dominant band. In conclusion, we have shown that T-cell monoclonality is a feature of the ulcers in both UJ and EATL and that the same clone is present in EATL and its associated inflammatory ulcers and in UJ and subsequently developing lymphoma.
 ...
PMID:Molecular analysis of T-cell clonality in ulcerative jejunitis and enteropathy-associated T-cell lymphoma. 925 Jan 61

A previously healthy 6-year-old boy developed symptoms of small intestinal obstruction and was found to have a large intraabdominal mass. At laparotomy the mass involved the jejunum and adjacent mesenteric lymph nodes, requiring resection. Microscopic and immunohistochemical studies demonstrated a T-cell non-Hodgkin's lymphoma, confirmed by finding clonal T-cell receptor-beta and -gamma gene rearrangements by Southern blot analysis. The immunophenotype of this lymphoma-CD3+CD4-CD8-CD56+TIA-1+ beta F1(-)-suggests that the tumor cells are cytotoxic natural killer (NK)-like T cells, probably of CD3+CD4-CD8- intraepithelial cell origin. Examination of the adjacent and distal small intestinal mucosa failed to show any significant pathologic change. This case was unusual because intestinal lymphomas in children are usually of B-cell origin and most commonly have small noncleaved cell morphology. Childhood intestinal T-cell lymphomas have not been the focus of specific study but appear to be rare. In adults, intestinal T-cell lymphomas often arise in the background of gluten-sensitive enteropathy (celiac disease). In contrast, this child had peripheral T-cell lymphoma, with NK-like T-cell features, in the small intestine with no clinical or histologic evidence of enteropathy.
 ...
PMID:Natural killer-like T-cell lymphoma in the small intestine of a child without evidence of enteropathy. 925 61

Primary gastrointestinal lymphoma comprises a group of distinctive clinicopathological entities. They may be of B or T-cell type. Intestinal T-cell lymphomas are much less common and include the entity: lymphomas T enteropathy-associated T-cell lymphoma, the most common, and T-cell lymphoma without features of enteropathy. The morphologic and immunologic findings suggest that derived from mucosal T lymphocytes population. Clinically, the patients were usually males with constitutional symptoms and acute perforation and/or obstruction of the small bowel. Their prognosis are very poor and tumor are very aggressive.
 ...
PMID:[Primary intestinal T lymphoma]. 959 39

Epstein-Barr virus is universally associated with endemic Burkitt's lymphoma (BL) and undifferentiated nasopharyngeal carcinoma and can be detected in a significant proportion of cases of Hodgkin's disease (HD) and peripheral T-cell lymphoma, but only rarely in sporadic B-NHL. The frequency of EBV-positivity in certain neoplasms shows important geographic variations. Both HD and sporadic BL from Latin America have shown higher rates of EBV-association than cases from Western countries. In T-NHL, the frequency of EBV-positivity is influenced by the site of the primary tumor and the phenotype of the neoplastic cells. Nasal and nasal-type T-NHL, which show a T/NK-cell phenotype with expression of CD56 are virtually always EBV-associated, whereas only a proportion of nodal, gastrointestinal and pulmonary T-NHL are EBV-infected. A recent investigation of primary intestinal lymphomas of Mexican origin demonstrated EBV-positivity in all examined cases of T-NHL and BL and a proportion of other B-NHLs. The presence of EBV was independent of the presence or absence of enteropathy. Two of 6 cases studied showed CD56 expression. The high rate of EBV-positivity independent of histologic subtype is in contrast to the low to intermediate rates of EBV-positivity found in cases of intestinal T-NHL from Western countries and indicates that geographic differences in the frequency of EBV-association of lymphoid neoplasms might also extend to a fraction of peripheral T-cell lymphomas.
 ...
PMID:Primary intestinal non-Hodgkin's lymphoma and Epstein-Barr virus: high frequency of EBV-infection in T-cell lymphomas of Mexican origin. 966 81

We report a case of enteropathy-associated T-cell lymphoma (EATL), diagnosed by small intestine and gastric biopsies, who presented with manifestations of hypocalcemia and malabsorption. Immunological assessment revealed increased expression levels of tumor necrosis factor system components and eotaxin, an observation that is consistent with the cytotoxic T-cell phenotype characteristic of EATL, and decreased numbers of circulating activated (CD8+CD38+ and CD4+CD25+) and suppressor (CD11b+) T cells, a feature which can contribute to lymphomagenesis in patients with celiac disease. The acute clinical presentation of the patient resolved with mineral and vitamin supplementation and a gluten-free diet. The novel immunological findings described are discussed in the context of a review of our current knowledge of the immunopathogenesis of celiac disease and associated intestinal neoplasia.
 ...
PMID:Enteropathy-associated T-cell lymphoma and its immunocarcinogenic correlates: case report and review of the literature. 975 48

Excluding mycosis fungoides, almost one third of T-cell lymphomas arise as primary tumors in extranodal sites, and these lymphomas are biologically different from their nodal counterparts. The revised European-American classification of lymphoid neoplasms and the World Health Organization classification have emphasized the importance of site in defining T-cell neoplasms and have included such new categories as hepatosplenic gamma delta T-cell lymphoma, subcutaneous panniculitis-like T-cell lymphoma, nasal and nasal-type T-cell lymphoma, enteropathy-type intestinal T-cell lymphoma, and primary cutaneous anaplastic large cell lymphoma. Although site is important, different lymphomas may occur at a particular location, and multiple parameters are required to define each type precisely. Cytologic features usually are not specific, and there are no morphologic correlates, such as follicular nodulation or plasmacytic differentiation in the B-cell system, to help define T-cell neoplasms. The T-cell system is biologically complex, consisting of populations with alpha beta and gamma delta receptors and helper and suppressor/cytotoxic phenotypes. In addition, NK cells resemble T cells in antigen expression, function, and patterns of disease, adding to the difficulty in defining T-cell and NK-cell neoplasms. Therefore, a complete workup with a combination of clinical, immunophenotypic, cytogenetic, and molecular genetic studies often is necessary to characterize these neoplasms. The role of each of these parameters in the diagnosis of T-cell and NK-cell neoplasms is discussed.
 ...
PMID:The role of morphologic features, phenotype, genotype, and anatomic site in defining extranodal T-cell or NK-cell neoplasms. 989 75

Gastrointestinal lymphomas comprise a group of distinctive clinicopathological entities 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. 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 inhibited 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 but high-grade diffuse large B-cell lymphomas may also arise de novo. Immunoproliferative small intestinal disease (IPSID) is a special form of MALT lymphoma characterized by synthesis of alpha heavy-chain immunoglobulin and a restricted geographic distribution. Other B-cell lymphomas that tend to arise in the gastrointestinal tract include mantle cell lymphoma, which presents as lymphomatous polyposis, Burkitt's lymphoma, and B-cell lymphomas associated with immunodeficiency states. 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.
 ...
PMID:Gastrointestinal lymphomas of T- and B-cell types. 1007 40

Natural killer (NK) cell lymphoma is a mass-forming neoplasm of putative NK cell lineage that typically appears in extranodal locations and has the following immunophenotype: CD2 positive, surface CD3 negative, cytoplasmic CD3 positive, and CD56 positive. We report a case of small-intestinal NK cell lymphoma that was originally diagnosed as an enteropathy-associated T-cell lymphoma based on paraffin immunohistochemistry. However, subsequent flow cytometric immunophenotyping of paracentesis fluid resulted in the correct diagnosis. We describe the case to illustrate the usefulness of this technique, which has not previously been described in such a case.
 ...
PMID:Natural killer cell lymphoma of the small intestine: diagnosis by flow cytometric immunophenotyping of paracentesis fluid. 1020 11

Loss of response to a gluten-free diet (refractory sprue) and ulcerative jejunitis are complications of celiac disease that may progress to enteropathy-associated T-cell lymphoma (EATL). Both conditions are characterized by the presence of a nonlymphomatous monoclonal T-cell population in the enteropathic mucosa. In EATL, a similar monoclonal population that shows clonal identity with the lymphoma itself is also present in the enteropathic mucosa. In this study we show that in all three circumstances the monoclonal T-cell population is constituted by cytologically normal, noninvasive intraepithelial T lymphocytes that share an identical aberrant immunophenotype with EATL. Patients with refractory sprue and/or ulcerative jejunitis are, therefore, suffering from a neoplastic T-cell disorder for which hematological treatment strategies need to be devised.
 ...
PMID:Mucosal intra-epithelial lymphocytes in enteropathy-associated T-cell lymphoma, ulcerative jejunitis, and refractory celiac disease constitute a neoplastic population. 1038 21

A 42 year old man presented with gluten-responsive coeliac disease and secondary pancreatic insufficiency. Subsequently his symptoms relapsed and repeat small intestinal biopsy showed villous atrophy and infiltration by leukaemic cells, despite continuation of a gluten-free diet. Serious causes of relapse and non-responsiveness in coeliac disease include enteropathy-associated T-cell lymphoma, ulcerative jejunitis and an end-stage hypoplastic mucosa. This is the first report of non-responsiveness due to infiltration by leukaemia.
 ...
PMID:A new cause of 'non-responsiveness' in coeliac disease? 1072 67


<< Previous 1 2 3 4 5 6 7 8 9 10 Next >>