1/8. A case of lymphoma-type alpha-chain disease.A 69-year-old man with a rare case of lymphoma-type alpha-chain disease was admitted to the hospital with marked cervical and inguinal lymph node swelling. Lymph node biopsy showed marked infiltration of plasma cells, plasmacytoid cells and immunoblastoid cells, alone or in combination. immunoelectrophoresis and immunoselection identified alpha-chain in the serum and urine. The site of alpha-chain synthesis was extensively studied in the whole body, and the immunoperoxidase technique eventually demonstrated the presence of alpha-chains in the cytoplasm of infiltrating malignant cells in the lymph nodes. No infiltrating malignant cells were found in other organs or tissues, including those of the digestive and respiratory tracts.- - - - - - - - - - ranking = 1keywords = digestive, tract (Clic here for more details about this article) |
2/8. Cytopathology of alpha chain disease involving the central nervous system and pleura.Alpha chain disease, a lymphomatous disorder characterized by the synthesis and secretion of an abnormal IgA immunoglobulin devoid of light chains, involves mainly the gastrointestinal tract. This paper presents the cytologic and histologic findings in two cases of alpha chain disease involving the central nervous system and pleura. Most of the cell populations in the cerebrospinal fluid (CSF) and pleural fluid resembled immature plasma cells (immunoblasts); many of these cells were degenerated, with well-preserved plasma cells seen more rarely. While the definitive diagnosis of alpha chain disease depends on immunochemical analysis of serum proteins, cytology can play a role by the identification of malignant cells in CSF and pleural fluid specimens. A positive staining of such cells by the methyl green pyronin reaction may permit the correct diagnosis to be suggested.- - - - - - - - - - ranking = 0.004466324973932keywords = tract (Clic here for more details about this article) |
3/8. 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 = 0.004466324973932keywords = tract (Clic here for more details about this article) |
4/8. A new form of alpha-chain disease with generalized lymph node involvement.An autopsy case of alpha-chain disease (ACD) clinically manifesting generalized lymph node swelling, slight splenomegaly and long-standing ichthyosiform skin eruptions, was reported. autopsy revealed systemic superficial and profound lymph node swelling and slight splenomegaly, but little or no tumorous lesion in any part of the alimentary tract or pulmonary tissue. The histologic picture of the lymph nodes showed a diffuse monomorphic plasmocytic lymphoma, and there was tumor cell infiltration in the spleen and bone marrow. immunohistochemistry demonstrated that the tumor cells contained IgA devoid of light chains, i.e. ACD protein. Immunoelectron microscopy revealed that this abnormal immunoglobulin was localized in the rough endoplasmic reticulum and perinuclear space. Persistent chronic inflammation with infiltration mainly of helper-inducer T cells were found in the skin and dermatopathic lymphadenopathy was confirmed in the lymph node biopsies. From these peculiar clinicopathological features, this case is considered to be a previously unknown form of ACD.- - - - - - - - - - ranking = 0.004466324973932keywords = tract (Clic here for more details about this article) |
5/8. 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 = 0.004466324973932keywords = tract (Clic here for more details about this article) |
6/8. 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 = 4keywords = digestive, tract (Clic here for more details about this article) |
7/8. Alpha-chain disease. Report of a case from taiwan.The occurrence of alpha-chain disease (alpha-CD) in a Chinese patient, a resident of taiwan, is reported. The patient's clinical features were characterized by severe malabsorption and ran a rapidly deteriorated course. Duodenal biopsy showed total villous atrophy with a heavy infiltration of the lamina propria by lymphoplasmacytic cells. Colonic biopsy revealed immunoblastic lymphoma. alpha-CD protein was demonstrated in the patient's serum and concentrated urine by the immunoselection method, and also detected in the cellular infiltrate by immunoperoxidase technique. Postmortem examination disclosed that the lymphoma involved not only the entire gastrointestinal tract and mesenteric nodes, but also the retroperitoneal and peripheral lymph nodes. Immunoperoxidase studies support the concept of a common clonal origin of the lymphoplasmacytic proliferation and immunoblastic lymphoma in alpha-CD.- - - - - - - - - - ranking = 0.004466324973932keywords = tract (Clic here for more details about this article) |
8/8. immunoproliferative small intestinal disease (IPSID) in thailand.immunoproliferative small intestinal disease (IPSID) is prevalent in the mediterranean region and in many Third World countries but is rare in Southeast asia. Between 1980-1990, 4 cases of IPSID were admitted to Ramathibodi Hospital, Bangkok. Three were males and the mean age was 32 /- 20.2 years. All patients presented with chronic diarrhea of 7 months to 6 years duration, and weight loss of 15 to 31 kg. All were malnourished, three cachectic, and one patient showed growth retardation. Intestinal parasites were found in all cases: two had multiple infections and three had uncommon protozoal infections (coccidium, cryptosporidium). barium radiographs revealed intestinal mucosal fold thickening with malabsorption pattern in all cases. Alpha chain IgA was detected in one patient. The remainder underwent exploratory laparotomy and the histological finding was of plasma lymphocytic infiltration of the small intestinal mucosa. All patients responded to oral tetracycline with complete remission occurring in one case. During the follow-up period, 3 cases had progressive retractable clinical courses but all died 2 to 5 years after the diagnosis. The causes of death in these patients were secondary bacterial infection (1 case), intestinal tuberculosis (1 case), fungal infection (1 case) and immunoblastic sarcoma in another case. The results of this study confirm the occurrence of IPSID in thailand. IPSID responds to oral antibiotic therapy and complete remission may be achieved during the early reversible benign phase, thus an awareness of its occurrence is of clinical importance.- - - - - - - - - - ranking = 0.004466324973932keywords = tract (Clic here for more details about this article) |