1/13. ataxia in the setting of complicated enteropathy: double jeopardy.The differential diagnosis of subacute onset ataxia in the setting of enteropathy is wide. A 54 year old patient with a pancerebellar syndrome and known ulcerative jejunoileitis is described. Small bowel biopsy showed evidence of enteropathy associated T cell lymphoma and subsequent neuropathological analysis and immunophenotyping confirmed metastasis of this tumour to the cerebellum. The presence of anti-gliadin antibodies and MRI evidence of a more longstanding process suggested additional immunologically mediated cerebellar dysfunction. Lymphomatous involvement of the CNS is rare in patients with complicated enteropathies, and has not been previously reported to involve the cerebellar parenchyma. This diagnostic possibility should be borne in mind before attributing cerebellar dysfunction in patients with the coeliac related enteropathies to nutritional compromise or immunological dysfunction (gluten ataxia) alone.- - - - - - - - - - ranking = 1keywords = enteropathy (Clic here for more details about this article) |
2/13. Synchronous collagenous sprue and enteropathy-type T cell lymphoma: variants of the same disease.A 64-year-old man with treated hypothyroidism had 10 months of diarrhea, abdominal pain, anorexia and recent involuntary 13.6 kg weight loss. He presented to hospital with an acute abdomen that had a radiological correlate of free air under the diaphragm. He was diagnosed with a perforated mid-jejunum due to an ulcerated enteropathy-type T cell lymphoma (ETL), complicating collagenous sprue and cryptic celiac disease. polymerase chain reaction verified monoclonal gamma- and beta-T cell receptor gene rearrangements in the neoplasm. He had a complete resolution of symptoms when treated with a gluten-free diet in the postoperative period. This is apparently the first report describing collagenous sprue and ETL as synchronous lesions. Because atypical CD8 lymphocytes are in both the collagenous sprue epithelium and ETL, the implication is that collagenous sprue is a noninvasive component of the ETL.- - - - - - - - - - ranking = 0.83333333333333keywords = enteropathy (Clic here for more details about this article) |
3/13. capsule endoscopy findings in celiac disease associated enteropathy-type intestinal T-cell lymphoma.capsule endoscopy is a new technology developed to investigate diseases of the small intestine. It has been shown to be superior to current modalities such as small-bowel radiography and enteroscopy. We describe a patient with long-standing celiac disease who presented with abdominal pain, diarrhea, and weight loss, after many years on a gluten-free diet. The symptom complex and results from small-bowel radiography and computerized tomography raised concern about progression to lymphoma, and ultimately a laparoscopy and small-bowel resection were done for diagnosis. A capsule endoscopy was performed to assess the extent of the patient's enteropathy-type intestinal T-cell lymphoma after three cycles of chemotherapy. We report the first use of capsule endoscopy in the setting of celiac disease associated enteropathy-type intestinal T-cell lymphoma. These endoscopic findings are correlated with those from gross and microscopic pathology and barium small-bowel radiography.- - - - - - - - - - ranking = 1keywords = enteropathy (Clic here for more details about this article) |
4/13. Disseminated enteropathy-type T-cell lymphoma: cauda equina syndrome complicating coeliac disease.A patient with coeliac disease developed a progressive cauda equina syndrome in the 2 months prior to his death, for which no cause could be identified. At post-mortem examination this proved to be due to disseminated enteropathy-type T-cell lymphoma. Although rare, disseminated enteropathy-type T-cell lymphoma should enter the differential diagnosis in any patient with coeliac disease developing new neurological signs.- - - - - - - - - - ranking = 1keywords = enteropathy (Clic here for more details about this article) |
5/13. Ulcerative jejunoileitis and enteropathy-associated t-cell lymphoma.Ulcerative jejunoileitis and enteropathy-associated t-cell lymphoma are rare conditions described in patients with refractory coeliac disease. Ulcerations affect the small bowel and are unrelated to drugs, ischaemia, infections or other known causes. We describe a female patient with an unclassified enteropathy who experienced several episodes of jejunoileal ulcerations. Several resections of the small bowel segments were necessary. The repetitive ulcerations were either from cytotoxic T cells, the patient developed a T-cell lymphoma, and malignant cells could be detected at the bottom of the ulcers, or from acid-producing cells in areas of gastric metaplasia. Two mechanisms might thus be responsible for the occurrence of repetitive ulceration, and require different treatment strategies. The patient is currently being treated with proton pump inhibitors, oral steroids and parenteral nutrition.- - - - - - - - - - ranking = 1keywords = enteropathy (Clic here for more details about this article) |
6/13. T-lymphoblastic lymphoma arising in the small intestine.The authors report the clinical, pathological and immunological features of a case of T-lymphoblastic lymphoma presenting with protein-losing enteropathy. There was extensive multifocal involvement of the duodenum, jejunum and ileum. The mediastinum was not enlarged; the peripheral blood picture and bilateral bone marrow trephine biopsies were unremarkable. The tumor cells were positive for terminal deoxynucleotide transferase, CD3, CD2, CD7 and CD10; they were negative for CD1, CD5, CD4, CD8 and HLA-DR. The immunophenotype was that of an immature thymic T-cell. Monocytic and B-cell markers were negative. Despite initial dose reduction in chemotherapy, the patient still developed massive intestinal hemorrhage and succumbed 2 wks after treatment. Postmortem examination confirmed absence of thymic involvement. The overall picture strongly suggests a primary intestinal origin of this T-lymphoblastic lymphoma which contradicts the conventional wisdom that T-lymphoblastic lymphoma arises in the thymus from primitive cortical lymphocytes before rapidly disseminating.- - - - - - - - - - ranking = 0.16666666666667keywords = enteropathy (Clic here for more details about this article) |
7/13. Protein-losing enteropathy in acquired immunodeficiency syndrome due to intestinal Kaposi's sarcoma.patients with the acquired immunodeficiency syndrome (AIDS) often have hypoalbuminemia. We report the case of a patient with AIDS in whom marked hypoalbuminemia developed due to a protein-losing enteropathy caused by small intestinal Kaposi's sarcoma--an entity not previously reported in AIDS. The patient presented with ankle edema, pleural effusions, and a decrease in albumin from 3.0 g/dL (30 g/L) to 1.7 g/dL (17 g/L) over one month. Protein-losing enteropathy was confirmed by a marked elevation in fecal alpha-1 antitrypsin, and extensive evaluation of the gastrointestinal tract revealed the source to be small intestinal Kaposi's sarcoma. A protein-losing enteropathy should be considered when hypoalbuminemia is encountered in a patient with AIDS.- - - - - - - - - - ranking = 1.1666666666667keywords = enteropathy (Clic here for more details about this article) |
8/13. Presence of clonal T-cell receptor gene rearrangements provides evidence of widespread intramucosal intestinal T-cell lymphoma.Intestinal T-cell lymphoma (ITCL) is a rare type of extranodal lymphoma derived from intraepithelial T-cells and generally exhibits macroscopically evident ulcerated lesions of the bowel wall composed of pleomorphic tumor cells. We report immunophenotypic and molecular genetic findings in an unusual small-sized intramucosal type of ITCL observed in a 74-year-old man presenting with enteropathy. Biopsies obtained from duodenum and jejunum showed a conspicuous accumulation of small-sized lymphoid cells forming intraepithelial clusters. The predominantly intraepithelial spread was accompanied by a spilling over to the lamina propria but not to deeper layers of the duodenal and jejunal wall, thus resulting in a purely intramucosal manifestation. This growth pattern and the immunophenotypic profile (CD3 , CD4-, CD8 , CD103 ) suggested that the lesion may fall in the spectrum of ITCL. However, since the lymphoid cells cytomorphologically lacked neoplastic features and showed a small growth fraction. a reliable diagnosis of malignant lymphoma could not be established by histological and immunohistochemical methods. In this case a tissue-based polymerase chain reaction (PCR) analysis of T-cell receptor (TCR) beta and gamma gene rearrangements proofed to be essential in confidently distinguishing multifocal monoclonal intramucosal T-cell lymphocytosis from an intense reactive inflammatory infiltrate in celiac disease (CD). Our observation highlights that detection of clonal rearrangements of TCR genes is particularly useful in detecting ITCL composed of cytomorphologically innocuous T-cells because histologic diagnosis in such cases is at best presumptive.- - - - - - - - - - ranking = 0.16666666666667keywords = enteropathy (Clic here for more details about this article) |
9/13. enteropathy-associated t-cell lymphoma: a case report with radiographic and computed tomography appearance.We report a case of enteropathy-associated t-cell lymphoma (EATL) of the jejunum in a 56-year-old man. The patient suffered for several years from nonspecific abdominal complaints, with no clinical evidence of malabsorption. The patient underwent extensive imaging procedures including barium meal and computed tomography. Computed tomography of the abdomen showed small mesenteric lymph nodes and an area of intestinal wall thickening. barium meal demonstrated a short jejunal stricture. histology revealed lymphoma of the jejunum, with microscopic changes distant from the lesion consistent with celiac disease. The spectrum of EATL ranges from patients with frank celiac disease, to patients with only immunohistochemical evidence of celiac disease, who develop small bowel lymphoma.- - - - - - - - - - ranking = 0.16666666666667keywords = enteropathy (Clic here for more details about this article) |
10/13. Natural killer-like T-cell lymphoma in the small intestine of a child without evidence of enteropathy.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.- - - - - - - - - - ranking = 1keywords = enteropathy (Clic here for more details about this article) |
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