1/6. 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) |
2/6. 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) |
3/6. 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 = 0.88888888888889keywords = sprue (Clic here for more details about this article) |
4/6. Obstructive jaundice caused by enteropathy-associated t-cell lymphoma in a patient with celiac sprue.patients with celiac sprue carry a considerable risk of gastrointestinal malignancies; in particular, non-Hodgkin's lymphoma. These malignancies represent the most serious complications of celiac disease. Commonly, patients present with deteriorating symptoms of the underlying disease, which makes an early diagnosis difficult. We report a patient with a 13-year history of celiac sprue presenting with painless jaundice and a Courvoisier gallbladder. Abdominal computed tomography (CT) scan showed thickening of the duodenal wall, suggesting a neoplastic infiltration of the papilla of Vater, causing biliary obstruction. Biopsies taken on endoscopy revealed enteropathy-associated t-cell lymphoma of the duodenum. Biliary obstruction is a rare clinical finding in enteropathy-associated t-cell lymphoma. To our knowledge, this is the first reported case of this unusual manifestation in celiac disease.- - - - - - - - - - ranking = 0.66666666666667keywords = sprue (Clic here for more details about this article) |
5/6. Multimodal immunosuppressant therapy in steroid-refractory common variable immunodeficiency sprue: a case report complicating cytomegalovirus infection.Immunodeficient patients can develop malabsorption, mimicking celiac disease clinically and histologically. Such individuals may also occasionally require immunosuppressive therapy for autoimmune disorders. We have identified a patient with common variable immunodeficiency (CVID)-associated sprue complicated by duodenal cytomegalovirus (CMV) infection following corticosteroid and ancillary immunomodulatory therapy. ganciclovir and a modification of the immunosuppressant regimen improved both clinical symptoms and villous atrophy. To our knowledge, this is original documentation of duodenal CMV infection secondary to immunomodulatory therapy for steroid-refractory CVID-sprue.- - - - - - - - - - ranking = 0.66666666666667keywords = sprue (Clic here for more details about this article) |
6/6. Oral T-cell lymphoma associated with celiac sprue. A case report.Celiac sprue, also called nontropical sprue, is a malabsorption syndrome with symptoms that are triggered mainly by gluten ingestion. Non-Hodgkin's lymphoma of the gastrointestinal tract has been reported in patients with a long history of celiac sprue. This case report describes the occurrence of primary oral lymphoma in such a patient. This case is unusual because lymphomas associated with celiac sprue usually develop in the small intestine or in the stomach, and primary lymphoma in the mouth is itself uncommon.- - - - - - - - - - ranking = 0.88888888888889keywords = sprue (Clic here for more details about this article) |