1/16. immunoproliferative small intestinal disease: case report and literature review.immunoproliferative small intestinal disease (IPSID) is a subtype of lymphoma of mucosa-associated lymphoid tissue. Notable for a high production of alpha-heavy chains, it is designated alpha-heavy-chain disease. IPSID is a debilitating disease that has a predilection for impoverished populations of developing countries. It has been documented primarily in subjects of Middle Eastern countries and thus was previously referred to as Mediterranean lymphoma. We report the case of a 42-year-old man from senegal who presented with chronic diarrhea, dehydration, and weight loss. The endoscopic, pathologic, and serologic findings before, during, and after treatment with fludarabine phosphate are presented. We review the literature concerning current concepts on the etiology, pathogenesis, and management of IPSID.- - - - - - - - - - ranking = 1keywords = mucosa (Clic here for more details about this article) |
2/16. immunoproliferative small intestinal disease: prolonged 30-year course without development of lymphoma.immunoproliferative small intestinal disease (IPSID) is mostly found in young adults of low socioeconomic class in developing countries. This condition is characterized by a dense lymphoplasmacytic infiltrate beneath the epithelium in the duodenal and proximal jejunal mucosa and in the mesenteric lymph nodes. In two thirds of cases, the involved lymphocytes elaborate an anomalous alpha-heavy chain protein. The etiology of this disease is unclear, although various parasitic, genetic, and toxic mechanisms have been proposed. Half of all IPSID patients will be found at diagnosis to have a concurrent intestinal B-cell lymphoma, and most of the remaining patients develop frank lymphoma within a few years. Although most reports of IPSID are from developing nations or indigent immigrant populations within Western countries, four cases of an IPSID-like condition have been documented in white women. Furthermore, although many IPSID patients progress to high grade indeterminate-type lymphoma within a few years of initial presentation, there have been occasional reports of long term survival without lymphomatous conversion. Here, we present an atypical case of IPSID--a california native who, though of Mexican heritage, had resided in the united states his entire life and did not belong to an indigent population. This patient had biopsy-proven IPSID that progressed over 30 yr but never exhibited lymphomatous conversion despite end stage intestinal stasis and recurrent obstruction, culminating in death. Our case calls into question some current assumptions about the prelymphomatous nature of this disease.- - - - - - - - - - ranking = 1keywords = mucosa (Clic here for more details about this article) |
3/16. Leukemic manifestation in a case of alpha-chain disease with multiple polypoid intestinal lymphocytic lymphoma.We describe a female aborigine from taiwan with alpha-chain disease associated with multiple polypoid intestinal lymphocytic lymphoma and leukemic manifestation. Initially, the patient experienced intermittent diarrhea, abdominal pain, and leukemic manifestation. No evidence of bone marrow involvement was found. Alpha-chain protein was demonstrated in the serum. Gastroendoscopy and a series of radiographs of the small intestine revealed multiple polypoid tumors involving the entire length of the small intestine. Duodenal biopsy showed diffuse lymphocytic lymphoma. Immunohistochemical staining of tumor samples revealed features typical of alpha-chain disease. cytogenetic analysis showed the same abnormal karyotypes of neoplastic clones in intestinal tumor cells and in circulating leukemic cells. The data suggest that alpha-chain disease can present initially with intestinal multiple polypoid lymphocytic lymphoma and leukemic manifestation without evidence of bone marrow involvement. The data also support the homing theory of lymphomas from mucosa-associated lymphoid tissue.- - - - - - - - - - ranking = 1keywords = mucosa (Clic here for more details about this article) |
4/16. immunoproliferative small intestinal disease associated with campylobacter jejuni.BACKGROUND: immunoproliferative small intestinal disease (also known as alpha chain disease) is a form of lymphoma that arises in small intestinal mucosa-associated lymphoid tissue (MALT) and is associated with the expression of a monotypic truncated immunoglobulin alpha heavy chain without an associated light chain. Early-stage disease responds to antibiotics, suggesting a bacterial origin. We attempted to identify a causative agent. methods: We performed polymerase chain reaction (PCR), dna sequencing, fluorescence in situ hybridization, and immunohistochemical studies on intestinal-biopsy specimens from a series of patients with immunoproliferative small intestinal disease. RESULTS: Analysis of frozen intestinal tissue obtained from an index patient with immunoproliferative small intestinal disease who had a dramatic response to antibiotics revealed the presence of campylobacter jejuni. A follow-up retrospective analysis of archival intestinal-biopsy specimens disclosed campylobacter species in four of six additional patients with immunoproliferative small intestinal disease. CONCLUSIONS: These results indicate that campylobacter and immunoproliferative small intestinal disease are associated and that C. jejuni should be added to the growing list of human pathogens responsible for immunoproliferative states.- - - - - - - - - - ranking = 1keywords = mucosa (Clic here for more details about this article) |
5/16. Plasma postheparin diamine oxidase in patients with small intestinal lymphoma.Diamine oxidase (DAO) is an enzyme located almost exclusively in villus tip enterocytes. Its plasma activity is enhanced by intravenous heparin which releases the enzymes from small bowel enterocytes into the blood. Plasma postheparin DAO (PHD) values have been shown to be significantly lower in patients with malabsorption and villous atrophy, thus suggesting that PHD reflects the mature enterocytic mass. In this study we have assayed PHD in five patients with small bowel lymphoma (two with immunoproliferative small intestinal disease [IPSID] and three with non-IPSID lymphoma) associated with malabsorption syndrome and small bowel mucosa atrophy. The PHD test was performed at diagnosis, after partial or complete remission induced by chemotherapy, and during the follow-up. The PHD values, very low at diagnosis (0.66 /- 0.12 U/ml), increased during chemotherapy and reached the normal range (greater than 3.7 U/ml) when complete remission occurred. The PHD values rapidly and consistently decreased whenever the disease relapsed. Our data indicate that in patients with small bowel lymphoma PHD test is a sensitive marker of small bowel mucosa damage and suggest that it could be useful in monitoring the recovery of mucosal lesions induced by chemotherapy.- - - - - - - - - - ranking = 3keywords = mucosa (Clic here for more details about this article) |
6/16. Immunoglobulin gene rearrangement in immunoproliferative small intestinal disease (IPSID).Analysis of dna from the mucosal tissue of three patients with immunoproliferative small intestinal disease (IPSID) and alpha chain disease, two of whom had early stage disease responsive to antibiotics, showed monoclonal heavy and light chain gene rearrangements in all cases. These findings suggest that IPSID is neoplastic even in its early stages, but that the neoplastic cells respond to normal stimuli. Monoclonal lymphoid populations could not be detected in circulating lymphocytes from these patients, which raises the possibility that the circulatory pathways of lymphocytes derived from human gut associated lymphoid tissue may not necessarily parallel those in experimental animals.- - - - - - - - - - ranking = 1keywords = mucosa (Clic here for more details about this article) |
7/16. Macroglobulinemia and small intestinal disease. A case report with review of the literature.Involvement of the gastrointestinal tract in macroglobulinemia is exceedingly rare. We describe a patient with IgM biclonal immunoglobulin disorder associated with diffuse lymphoplasmacytic infiltration of the small intestine. This chronic illness was characterized by diarrhea, steatorrhea, and intestinal pseudo-obstruction. Full-thickness biopsy specimens of the jejunum showed stunted and fused villi, giardiasis, and a widespread cellular infiltrate in the lamina propria extending through the muscularis mucosae into the submucosa and muscular layers. The infiltrate had a cytologically benign appearance that was shown to be polyclonal by immunochemical stains. Intestinal vacuolated plasma cells were occasionally observed in electron microscopic study. The patient has not developed the features of Waldenstrom's disease on a clinical follow-up of 14 years.- - - - - - - - - - ranking = 165.34841861262keywords = lamina propria, propria, lamina, mucosa (Clic here for more details about this article) |
8/16. Diffuse small intestinal lymphoid infiltration in nonimmunodeficient adults from Western europe.In two white adults born, raised, and living in central france and presenting with long-lasting malabsorption, massive and diffuse lymphoid infiltrate of the lamina propria associated with crypt scarcity was found along the whole small bowel. It was mostly composed of mature lymphocytes, focally mixed with plasma cells and reactive germinal centers. There was no evidence of celiac disease, systemic or intestinal immune deficiency or alpha-chain disease, overt lymphoid malignancy, or stagnant-loop syndrome. By immunofluorescence the infiltrate was constituted in 1 case of polyclonal B cells and, in the other, of a large majority of T11, T8, T10, and class II-positive T cells associated with a population of monotypic B cells. A gluten-free diet and parenteral nutrition proved ineffective. A dramatic and protracted clinical response was observed in both patients after the onset of oral tetracycline therapy, and still persists after 8 and 5.5 yr, respectively, together with morphologically unchanged small bowel infiltrate. These cases may be the equivalents, in people from Western developed countries, of the predominantly lymphocytic variety of the immunoproliferative small intestinal disease described in people from developing countries.- - - - - - - - - - ranking = 163.34841861262keywords = lamina propria, propria, lamina (Clic here for more details about this article) |
9/16. Massive plasma cell infiltration of the digestive tract. secretory component as the rate-limiting factor of immunoglobulin secretion in external fluids.A 29-yr-old Tunisian man had a clinical immunoproliferative small intestinal disease, different from alpha-chain disease. serum contained 52.5 mg/ml of polymeric immunoglobulin a (IgA). immunohistochemistry revealed a massive diffuse polyclonal IgA (99%)-plasma cell infiltration in the small bowel mucosa, with a smaller increase of IgA-producing cells in gastric and colonic mucosae. Secretory IgA levels were normal in jejunal and bronchoalveolar secretions. However, both fluids contained polymeric IgA devoid of secretory component, and free secretory component was absent. This suggests that secretory component was the limiting factor in transport of IgA in the secretions. A relative deficiency in secretory component, as compared with the huge supply of polymeric IgA, may have limited the secretory component-mediated active transport of IgA into secretions. This resulted in the appearance of high levels of polymeric IgA, unlinked to secretory component, both in serum and in the jejunal and bronchoalveolar fluids.- - - - - - - - - - ranking = 2keywords = mucosa (Clic here for more details about this article) |
10/16. Severe malnutrition associated with alpha-heavy chain disease: response to tetracycline and intensive nutritional support.A 20-yr-old black male was admitted with a 5-month history of profound weight loss and diarrhea. appetite and dietary intake had been remarkably well preserved up until the week before admission. The severity of his depletion was evidenced by a body weight of only 38% of standard, multiple electrolyte deficiencies, and reduced metabolic expenditure, protein turnover, protein synthesis, and pancreatic function. Immunological defects included diminished lymphocyte numbers, lymphocyte transformation, gamma-globulin concentration, and cell-mediated immunity. A diagnosis of alpha-heavy chain disease (alpha-HCD) was made on endoscopic duodenal biopsy and serology--lymphoma being excluded by scanning and laparotomy. Treatment consisted initially of intravenous nutrition (because of the extreme malnutrition, severe diarrhea, and malabsorption of fluid, electrolytes, carbohydrates, and fat) and oral tetracycline. Response was dramatic, with a doubling of body weight within 6 wk, and resolution of malabsorption. He was discharged on a normal diet and long-term oral tetracycline (250 mg/day), and at 1-yr follow-up, nutritional status and gut function were normal despite persistence of duodenal mucosal abnormalities and markers of alpha-HCD and bacterial overgrowth. These results suggest that the malabsorption initially identified in this patient was not due simply to the mucosal abnormalities that characterize alpha-HCD, but was more a consequence of the superimposition of nutrient maldigestion and absorption resulting from the extreme state of protein deficiency and its effects on gut and pancreatic function.- - - - - - - - - - ranking = 2keywords = mucosa (Clic here for more details about this article) |
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