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Query: UMLS:C0024523 (malabsorption)
 7,319 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Twenty-seven cases of primary peripheral T-cell lymphomas of the intestine (PTLI) were investigated. Seven patients had histories of malabsorption. The most frequent symptoms at presentation were weight loss, abdominal pain, and acute abdomen. The jejunum was the most common site of lymphoma and multifocal disease was found in 72% of the cases. Twenty-two patients (92%) presented with localized disease confined to the intestine and abdominal lymph nodes, only two patients had generalized disease. According to the pattern of lymphoma infiltration and the morphology of the uninvolved small intestinal mucosa, 21 cases were separated histologically into three categories; 1) enteropathy-associated T-cell lymphoma (EATCL, n = 9) showing predominant intramucosal lymphoma spread and villous atrophy of uninvolved mucosa with high density of intraepithelial lymphocytes (IEL), 2) EATCL-like lymphoma without enteropathy (EATCL-LLWE, n = 5) but with an infiltration pattern similar to EATCL, and 3) T-cell lymphoma without features of EATCL (Non-EATCL, n = 7). Distinctive features of EATCL were the high incidence of malabsorption states, multifocal intestinal disease in all cases, and the high frequency of intestinal recurrences. On frozen sections four of eight PTLI showed the phenotype CD3+ CD4- CD8- HML-1+, which is also expressed on a small subset of normal IEL. The morphologic and immunomorphologic findings suggest that the majority of PTLI is derived from mucosal T lymphocytes. This derivation may be responsible for certain biologic features, such as the preferential spread to and relapse of PTLI at small intestinal sites.
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PMID:Peripheral T-cell lymphomas of the intestine. 146

Celiac disease is defined as a GSE. The small intestinal histological appearance of villous atrophy with crypt hyperplasia, inflammatory cell infiltrate of the lamina propria, and epithelial cell abnormalities is characteristic but not pathognomonic of the disorder. Confirmation of the diagnosis depends on histological improvement when gluten is removed from the diet and deterioration following gluten reintroduction. The pathogenesis of celiac disease appears to require interaction between a number of factors both intrinsic (genetic susceptibility, activation of the immune system) and extrinsic (gluten susceptibility, activation of the immune system) and extrinsic (gluten and possibly other environmental factors). The diagnosis of GSE may be delayed or missed unless the clinician is aware of the broad clinical spectrum of disease presentation. Although celiac disease is widely perceived as a malabsorption syndrome of childhood, the diagnosis is increasingly being made for the first time in adult life. A significant number of patients have no GI symptoms whatsoever. Small intestinal biopsy through the endoscope is the initial and definitive investigation. Most patients show excellent clinical and histological response to a gluten-free diet. The commonest reason for poor response is continuing intentional or inadvertent gluten intake. A minority of patients develop complications, in particular intestinal malignancy, including enteropathy-associated T-cell lymphoma.
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PMID:Diagnosis and treatment of gluten-sensitive enteropathy. 240 97

The authors report the clinical, pathologic, and immunologic features of a case of jejunal cytotoxic/suppressor T-cell lymphoma associated with intractable malabsorption. Histologically, the tumor exhibited striking involvement of small bowel surface and glandular epithelium, and of epithelium in sites of disease dissemination. This epitheliotropism consisted of both cell clusters resembling Pautrier 's microabscesses and single cells within epithelium. Grossly, the jejunal mucosal fold pattern was completely obliterated by lymphoma which formed miliary nodules and multiple distinct tumor masses. Despite aggressive chemotherapy the patient developed widespread disease, and died 11 months after presentation. At autopsy, in addition to disseminated lymphoma, there was a notable activation of hematopoiesis evidenced by extensive extramedullary hematopoiesis and bone marrow hypercellularity. Many lymph nodes spared by the lymphoma showed a polyclonal proliferation of plasma cells and immunoblasts. In view of recent immunologic evidence that normal cytotoxic/suppressor T-cells selectively home to the gut surface epithelium, striking tumor cell epitheliotropism may be a morphologic marker for visceral lymphomas of cytotoxic/suppressor T-cell origin. This unique case broadens the clinical and morphologic spectrum of T-cell disorders.
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PMID:Epitheliotropic lymphoma of the small bowel. Report of a fatal case with cytotoxic/suppressor T-cell immunotype. 623 97

Mycosis fungoides (MF) is an uncommon T-cell lymphoma which characteristically involves the skin. Two patients with MF are described who developed fatal complications secondary to involvement of the gastrointestinal tract. One developed malabsorption due to small intestinal involvement; the other had a massive haemorrhage from an ulcerated nodule of tumour in the stomach. The potential for extracutaneous spread is discussed, and it is emphasized that bowel infiltration should be considered in any patient with MF who develops gastrointestinal symptoms or complications.
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PMID:Gastrointestinal complications of mycosis fungoides. 673 93

A 45-year-old woman with T-cell lymphoma is reported. Enlargement of the peripheral lymph nodes was not observed. Splenectomy was performed for hypersplenism and during the operation the mesenteric and retroperitoneal lymph nodes were found to be greatly enlarged. Three years after the diagnosis of lymphoma malabsorption syndrome developed. After 5 years sudden clinical deterioration appeared, with loss of reaction to effective treatment given as yet. Conversion of the predominating lymphocyte subpopulation from T to B cells was observed owing to repeatedly done monitoring of peripheral blood lymphocytes. The pattern of the newly developed lymphoma was that of a highly malignant B-cell proliferation. The possible mechanisms of the conversion discussed by the authors include: simultaneous proliferation of B and T populations of lymphocytes, B-cell lymphoma induction by chemotherapy, and infection by Epstein-Barr virus.
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PMID:[Transformation of type T lymphoma into type B lymphoma]. 697 55

We describe a 64-year-old woman with a malignant intestinal T-cell lymphoma who presented four years later with disabling osteomalacia and secondary hyperparathyroidism due to malabsorption. Only two years later, when the patient had developed fatty stools, flatulence and weight loss, diagnosis of gluten-sensitive enteropathy (GSE) was confirmed by small-intestine biopsy. This case report illustrates that in adults GSE can be oligosymptomatic for long periods. In cases of osteomalacia or rare intestinal T-cell lymphoma a detailed history of bowel movements, inspection of stools, quantification of fat excretion in stools and laboratory tests for malabsorption are recommended. Positive antibodies against gliadin, endomysium and reticulin may support the diagnosis of GSE. However, intestinal biopsy is necessary to verify the presence of GSE. In view of the unspecific histological changes, a follow-up biopsy is recommended in oligosymptomatic cases. Serial measurements of antibodies allow supervision of compliance for a diet strictly free of gluten. In addition, lactose containing milk products need to be restricted initially because of secondary lactase deficiency.
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PMID:[Gluten-sensitive enteropathy with intestinal T-cell lymphoma: an unusual cause of in disabling osteomalacia]. 748 45

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.
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PMID:Enteropathy-associated T-cell lymphoma: a case report with radiographic and computed tomography appearance. 917 68

Celiac disease is characterized by small intestinal damage with loss of absorptive villi and hyperplasia of the crypts, typically leading to malabsorption. In addition to nutrient deficiencies, prolonged celiac disease is associated with an increased risk for malignancy, especially intestinal T-cell lymphoma. Celiac disease is precipitated by ingestion of the protein gliadin, a component of wheat gluten, and usually resolves on its withdrawal. Gliadin initiates mucosal damage which involves an immunological process in individuals with a genetic predisposition. However, the mechanism responsible for the small intestinal damage characteristic of celiac disease is still under debate. Small intestinal biopsy with the demonstration of a flat mucosa which is reversed on a gluten-free diet is considered the main approach for diagnosis of classical celiac disease. In addition, IgA antibodies against gliadin and endomysium, a structure of the smooth muscle connective tissue, are valuable tools for the detection of patients with celiac disease and for therapy control. Incidence rates of childhood celiac disease range from 1:300 in Western Ireland to 1:4700 in other European countries, and subclinical cases detected by serological screening revealed prevalences of 3.3 and 4 per 1000 in Italy and the USA, respectively. IgA antibodies to endomysium are particularly specific indicators of celiac disease, suggesting that this structure contains one or more target autoantigens that play a role in the pathogenesis of the disease. However, the identification of the endomysial autoantigen(s) has remained elusive. We identified tissue transglutaminase as the unknown endomysial autoantigen. Interestingly, gliadin is a preferred substrate for this enzyme, giving rise to novel antigenic epitopes.
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PMID:Identification of tissue transglutaminase as the autoantigen of celiac disease. 921 95

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.
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PMID:Enteropathy-associated T-cell lymphoma and its immunocarcinogenic correlates: case report and review of the literature. 975 48

We describe a 52-year-old woman who presented with severe diarrhea, nausea, intermittent abdominal pain and weight loss of 18 kg within ten months. Jejunal and duodenal ulcers were detected by endoscopy and multiple biopsies revealed villous atrophy of the jejunum. However, neither gliadin nor endomysium antibodies were detected and no clinical and histological improvement was achieved after gluten withdrawal. Despite strong clinical suspicion for intestinal lymphoma many unrevealing biopsies were done. The patient developed intermittent septic fever and diagnostic laparotomy revealed jejunal perforation. Partial jejunal resection was performed and histology confirmed the diagnosis of an intestinal T-cell lymphoma without celiac disease. Malabsorption and all intestinal ulcers disappeared during the course of chemotherapy (six cycles CHOP) and the patient recovered remarkably.
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PMID:Malabsorption with progressive weight loss and multiple intestinal ulcers in a patient with T-cell lymphoma. 1045 10


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