1/5. 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 = 1keywords = sprue (Clic here for more details about this article) |
2/5. collagenous sprue associated with an extensive T-cell lymphoma.A 79-year-old woman developed collagenous sprue, a rare small intestinal mucosal disorder. Later, extensive T-cell lymphoma was documented, a neoplasm known to complicate celiac disease. Although the precise relationship of collagenous sprue to celiac disease has been debated and remains controversial, the findings here provide additional evidence that collagenous sprue and celiac disease are closely linked. In the past, long-term survival with collagenous sprue may have been compromised due to severe pan-malabsorption. With improved treatment measures, including modern nutritional support, it is likely that there will be an increased opportunity in future for improved appreciation of the complications of collagenous sprue, specifically, lymphoma.- - - - - - - - - - ranking = 1keywords = sprue (Clic here for more details about this article) |
3/5. T-cell intestinal lymphoma associated with celiac sprue.A patient with a long history of celiac sprue developed a pleomorphic intestinal lymphoma. Cell suspensions of tumor cells and immunoperoxidase labeling of cell surface antigens in frozen tissue sections clearly showed the lymphoma to be of T-cell origin, despite the presence of other nontumor cells bearing enzyme markers of histiocytes. These findings may have important implications on the cellular origin of lymphomas developing in patients with celiac sprue.- - - - - - - - - - ranking = 0.66666666666667keywords = sprue (Clic here for more details about this article) |
4/5. Multifocal small bowel lymphoma and latent celiac sprue.Malignant small intestinal lymphoma may complicate or antedate clinical recognition of celiac sprue, a disorder becoming increasingly diagnosed as a subclinical or occult disease. A 73-yr-old woman with previously resected jejunoileal lymphoma and normal proximal small bowel biopsy specimens was given a high-gluten diet containing 40 g of added gluten daily for 4 wk. This caused small intestinal biopsy abnormalities typical of celiac sprue; the abnormalities resolved 6 wk later with a gluten-free diet. This indicates that latent celiac sprue may be present in some patients with lymphoma and suggests that the association of celiac sprue and lymphoma may be more frequent than is currently appreciated.- - - - - - - - - - ranking = 0.88888888888889keywords = sprue (Clic here for more details about this article) |
5/5. Small bowel malignant lymphoma complicating celiac sprue and the mesenteric lymph node cavitation syndrome.Malignant small intestinal lymphoma may complicate or antedate clinical recognition of celiac sprue. However, histologic diagnosis of lymphoma is made especially difficult in the presence of small bowel ulceration. A 70-yr-old man with celiac sprue and a history of dermatitis herpetiformis was initially seen for recurrent diarrhea; panmalabsorption with steatorrhea and protein-losing enteropathy were documented. Subsequent studies showed ectopic gastric mucosa in the small bowel, hyposplenism with mesenteric lymph node cavitation, and small bowel erosions and ulceration. Despite strong clinical suspicion for more than 2 yr, only 1 of 88 small bowel biopsy specimens was positive for lymphoma. At autopsy, shortly after histologic diagnosis of lymphoma, extensive small bowel involvement and infiltration were observed. This is the first report of lymphoma complicating the recently described nonneoplastic lymphoreticular syndrome associated with celiac sprue characterized by splenic atrophy and mesenteric lymph node cavitation.- - - - - - - - - - ranking = 0.77777777777778keywords = sprue (Clic here for more details about this article) |