1/38. Natural killer cell lymphoma of the small intestine: diagnosis by flow cytometric immunophenotyping of paracentesis fluid.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.- - - - - - - - - - ranking = 1keywords = enteropathy (Clic here for more details about this article) |
2/38. Very late onset small intestinal B cell lymphoma associated with primary intestinal lymphangiectasia and diffuse cutaneous warts.As only a handful of lymphoma cases have been reported in conjunction with primary intestinal lymphangiectasia, it is not yet clear if this association is merely fortuitous or related to primary intestinal lymphangiectasia induced immune deficiency. We report on two female patients, 50 and 58 years old, who developed small intestinal high grade B cell lymphoma a long time (45 and 40 years, respectively) after the initial clinical manifestations of primary intestinal lymphangiectasia. They presented with a longstanding history of fluctuating protein losing enteropathy, multiple cutaneous plane warts, and markedly dilated mucosal and submucosal lymphatic channels in duodenal biopsies. One had a large ulcerated tumour of the proximal ileum and the other diffuse ileal infiltration. In both, histological examination showed centroblastic high grade B cell lymphoma associated with duodenojejuno-ileal mucosal and submucosal lymphangiectasia. They were subsequently successfully treated with surgery and postoperative chemotherapy (AVmCP: adriamycin, cyclophosphamide, Vm26, and prednisolone), and chemotherapy alone (PACOB: adriamycin, cyclophosphamide, vincristine, bleomycine, and prednisolone), respectively. A three year follow up in both cases showed persistent diffuse lymphangiectasia without evidence of lymphoma. The present findings support the hypothesis that primary intestinal lymphangiectasia is associated with lymphoma development.- - - - - - - - - - ranking = 1keywords = enteropathy (Clic here for more details about this article) |
3/38. Refractory sprue syndrome with clonal intraepithelial lymphocytes evolving into overt enteropathy-type intestinal T-cell lymphoma.INTRODUCTION: Recently, patients with refractory sprue have been shown to contain a clonal proliferation of phenotypically abnormal intraepithelial lymphocytes in their intestine. Whether this signifies early enteropathy-type intestinal T-cell lymphoma (EITCL) or a reactive condition is not clear. We report on a patient presenting with the findings of refractory sprue who subsequently developed overt EITCL. MATERIAL AND methods: Duodenal biopsies from 1997 (refractory sprue) and duodenal and jejunal biopsies from 1998 (intestinal T-cell lymphoma) were compared by immunohistochemistry and PCR for the detection of T-cell receptor (TCR)-gamma gene rearrangements. Clonal PCR products were sequenced. RESULTS: The duodenal biopsies from both 1997 and 1998 and the jejunal tumor biopsy showed villus atrophy and an increase of intraepithelial lymphocytes with an abnormal immunophenotype (CD3 , CD4-, CD8- and TCR-beta-). In all duodenal specimens including the one from 1997, and the jenunal tumor biopsy, an identical clonal amplificate was detected by enzymatic amplification of the TCR-gamma gene. CONCLUSION: These data suggest that refractory sprue containing a clonal proliferation of phenotypically abnormal intraepithelial lymphocytes may represent an early manifestation of EITCL. The detection of immunohistochemical negativity for several antigens normally found on intraepithelial lymphocytes such as CD8 or the TCR-beta chain in combination with clonal T-cell populations by PCR may be helpful in identifying refractory sprue with a malignant transformation.- - - - - - - - - - ranking = 5keywords = enteropathy (Clic here for more details about this article) |
4/38. Enterotherapy associated T-cell lymphoma: a case report and literature review.T-cell lymphoma of the intestine is a form of extranodal, non-Hodgkins lymphoma. Enteropathy associated T-cell lymphoma (EATCL) is a unique form of T-cell lymphoma involving the gastrointestinal tract. This disorder is rare and there is no consensus on guidelines for diagnosis or treatment. CASE: This 47 year old patient presented to the hospital with increasing abdominal girth and swelling of the lower extremities over the four days prior to admission. He complained of dyspnea on exertion, anorexia, nausea and vomiting, 40 pound weight loss over half a year, accompanied by intermittent diarrhea. On physical examination, the patient was found to have anasarca, an abdominal fluid wave with shifting dullness, mild abdominal tenderness, marked pitting edema of the extremities, and necrotic toes. A small bowel follow through revealed an abnormality in the mid-jejunum and a colonoscopy revealed two erythematous lesions near the cecum. Subsequent esophagoduodenoscopy and enteroscopy identified multiple white patchy lesions in the proximal jejunum. Biopsies were consistent with a T-cell lymphoma. The patient underwent a partial small bowel resection. Biopsies of the jejunum had ulceration and necrosis with a lymphomatous infiltrate. The lymphocytes were found to be a monoclonal CD2 positive T-cell population. A bone marrow biopsy was unremarkable. Enteropathy associated T-cell lymphoma is a rare, and this may be the first reported case of EATCL presenting as anasarca. DISCUSSION: The etiology and pathophysiology of intestinal lymphomas, particularly enteropathy associated T-cell lymphoma, is reviewed. CONCLUSION: EATCL is a rare and unique form of lymphoma without an exact treatment regimen which carries a high mortality rate.- - - - - - - - - - ranking = 1keywords = enteropathy (Clic here for more details about this article) |
5/38. Primary CD56 positive lymphomas of the gastrointestinal tract.BACKGROUND: Primary CD56 positive lymphoma of the gastrointestinal (GI) tract is rare. Genotypically, these tumors can be classified into natural killer (NK)-like T-cell lymphoma or NK cell lymphoma by the presence or absence of T-cell receptor (TCR) gene rearrangement. However, they have a considerable degree of morphologic and immunophenotypic overlap, making a definitive diagnosis difficult. methods: The clinicopathologic features of three patients with primary CD56 positive lymphoma of the small and large bowel are presented. This is followed by a review of the English literature from 1966 to the present. RESULTS: All patients had CD56 positive/CD3epsilon positive disease on paraffin section. Two patients were positive for Epstein-Barr virus-encoded early nuclear RNAs (EBER) according to in situ histochemistry results and were negative for TCR gene rearrangement, consistent with primary NK lymphoma of the GI tract. The other patient was EBER negative with rearranged TCR, consistent with NK-like T-cell lymphoma. There was no clinical or histologic evidence of enteropathy in any of the patients. The major presenting symptoms included fever, weight loss, and intestinal perforation. All patients died between 1 week and 6 months after diagnosis despite undergoing surgery and intensive chemotherapy. CONCLUSIONS: These results, together with a literature review, suggest that primary NK cell lymphoma of the GI tract may be considered a distinct clinicopathologic entity. Both primary NK and NK-like T-cell lymphoma pursue an aggressive clinical course. EBER and TCR gene rearrangement are useful in distinguishing NK cell lymphoma from NK-like T-cell lymphoma, particularly when frozen tissue is not available for immunophenotyping.- - - - - - - - - - ranking = 1keywords = enteropathy (Clic here for more details about this article) |
6/38. Intraabdominal lymphangiomyoma in an infant with protein-losing enteropathy and hemihypertrophy.lymphangiomyoma is an extremely rare tumor occurring exclusively in women of reproductive age. The tumor is characterized by proliferation of immature smooth muscle along the lymphatic vessels of the abdomen, thorax and lung. Although lymphangiomyoma has been reported in a young girl and a girl infant, none has been reported in boys. We report herein a case of lymphangiomyoma in a two-year-old boy. The unusual presentation in this patient was that the tumor arose from the small bowel mesentery without any evidence of lung involvement. The tumor was extirpated and lymphangiomyomatosis was confirmed pathologically.- - - - - - - - - - ranking = 4keywords = enteropathy (Clic here for more details about this article) |
7/38. iron deficiency anemia as the sole symptom of small intestine carcinoma.BACKGROUND: The isolated longstanding hypochromic hyposideremic anemia can be a unique symptom of the jejunal tumor. CASE REPORT: The authors present a case of 43-year-old woman with small intestine cancer, which manifested as longstanding anemia, decreased serum iron and remained undiagnosed over a period of several years. Special attention has been paid to the problem of adequate diagnostic procedure for disclosing the latent small intestine tumor.- - - - - - - - - - ranking = 2.5348403082483E-6keywords = aid (Clic here for more details about this article) |
8/38. The utility of Tc-99m dextran in the diagnosis and identification of melanoma metastases responsible for protein-losing enteropathy.Protein-losing enteropathy is an uncommon syndrome of excessive loss of protein via the gastrointestinal mucosa. Tc-99m dextran is a tracer ideally suited for diagnosis and localization. The authors report a case of melanoma mestastases to the small bowel that were causing protein-losing enteropathy. These were diagnosed and localized using Tc-99m dextran, leading to a curative resection.- - - - - - - - - - ranking = 6keywords = enteropathy (Clic here for more details about this article) |
9/38. Chromosomal gains at 9q characterize enteropathy-type T-cell lymphoma.Genetic alterations in enteropathy-type T-cell lymphoma (ETL) are unknown so far. In this series, 38 cases of ETL were analyzed by comparative genomic hybridization (CGH). CGH revealed chromosomal imbalances in 87% of cases analyzed, with recurrent gains of genetic material involving chromosomes 9q (in 58% of cases), 7q (24%), 5q (18%), and 1q (16%). Recurrent losses of genetic material occurred on chromosomes 8p and 13q (24% each), and 9p (18%). In this first systematic genetic study on ETL, chromosomal gains on 9q (minimal overlapping region 9q33-q34) were found to be highly characteristic of ETL. fluorescence in situ hybridization analysis on four cases of ETL, using a probe for 9q34, indicated frequent and multiple gains of chromosomal material at 9q34 (up to nine signals per case). Among 16 patients with ETL who survived initial disease presentation, patients with more than three chromosomal gains or losses (n = 11) followed a worse clinical course than those with three or less imbalances (n = 5). The observation of similar genetic alterations in ETL and in primary gastric (n = 4) and colonic (n = 1) T-cell lymphoma, not otherwise specified, is suggestive of a genetic relationship of gastrointestinal T-cell lymphomas at either localization.- - - - - - - - - - ranking = 5keywords = enteropathy (Clic here for more details about this article) |
10/38. Enteropathy-type T-cell lymphoma showing repeated small bowel rupture and refractoriness to chemotherapy: a case report.The majority of gastrointestinal lymphomas arise in the stomach, whereas lymphomas occurring in the intestine are relatively rare and a limited fraction of them show the T-cell phenotype with clinical manifestations similar to de novo celiac disease. Enteropathy-type T-cell lymphoma is extremely rare in japan, presumably owing to the very low incidence of celiac disease among the Japanese population. Here, we report a 66-year-old Japanese male who was diagnosed as having enteropathy-type T-cell lymphoma preceded by diarrhea and intermittent bloody stool for over 1 year. He was admitted to our hospital as an emergency case because of panperitonitis due to intestinal perforation. After immediate partial small-bowel resection, cyclophosphamide, doxorubicin, vincristine and prednisone (CHOP) chemotherapy was started. However, the disease was highly refractory and was exacerbated with leukemic transformation. Subsequent salvage chemotherapy could not be completed because of the formation of spontaneous jejuno-abdominal fistula, followed by fatal septic shock. Particular attention should be paid to the peculiar clinical manifestations of enteropathy-type T-cell lymphoma such as malnutrition, frequent intestinal perforation and refractoriness to chemotherapy.- - - - - - - - - - ranking = 2.0000025348403keywords = enteropathy, aid (Clic here for more details about this article) |
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