1/14. Unusual presentation of graft-versus-host disease in pediatric liver transplant recipients: evidence of late and recurrent disease.Graft-versus-host disease (GvHD) is a multi-organ disease caused by mature donor T cells that are activated by alloantigens expressed by the host antigen-presenting cells. GvHD has been reported after solid organ transplantation with two principal presentations: humoral and cellular. In the cellular type of GvHD after liver transplantation the symptoms are identical to the GvHD after bone marrow transplant, except that the liver is spared because it lacks host antigens. We have described three cases of intestinal GvHD after pediatric liver transplant with an unusual recurrent late presentation in two patients. Two patients were female, and their age at the time of transplant was 8 and 9 months, respectively, and one was an 8-month-old male. They all received reduced liver allografts of identical blood type from three different donors. One patient received two doses of donor bone marrow cell infusion. Two patients received double immunosuppressive therapy constituted by tacrolimus at a dose of 0.05 mg/kg p.o. b.i.d. and steroids 10 mg p.o. daily. One patient received a triple drug immunosuppression with tacrolimus (0.05 mg/kg p.o. b.i.d.), steroids (10 mg p.o. daily) and mycophenolate mofetil (125 mg p.o. b.i.d.). diagnosis of intestinal GvHD was confirmed histologically on intestinal biopsies performed at the time of presentation of the clinical symptoms or at autopsy.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
2/14. Identical T-cell expansions in the colon mucosa and the synovium of a patient with enterogenic spondyloarthropathy.Intestinal T lymphocytes activated by antigen are suspected to play a key role in enterogenic spondyloarthropathies (SpA). Therefore, we aimed to identify and functionally characterize T-cell clones that are coexpanded in the intestinal mucosa and the synovium. colon, peripheral blood, and synovium of a patient with enterogenic SpA were screened for clonal T-cell expansions by TCRB-CDR3 length analysis and sequencing. T-cell clones expanded in vivo were isolated from archived synovial cells by targeted T-cell cloning and characterized for phenotype, cytokine production, and antigen specificity. The synovial TCRBV18( ) T-cell repertoire of the patient was dominated by 2 CD8( ) T-cell clones using related CDR3. Both clones were expanded throughout the colon and were present in the peripheral blood. Upon in vitro stimulation with PDB/ionomycin, they showed predominantly interferon gamma and interleukin (IL)-4 but also tumor necrosis factor alpha and IL-10 production and did not specifically lyse autologous T-cell blasts, B-cell lines, or other autologous or allogeneic target or CD1d-transfected cells. These findings strongly suggest that T lymphocytes activated by antigen in the intestinal mucosa contribute to joint inflammation in enterogenic SpA by recognition of antigens specific for the inflamed synovium.- - - - - - - - - - ranking = 1.3333333333333keywords = antigen (Clic here for more details about this article) |
3/14. Galactomannan and polymerase chain reaction for the diagnosis of primary digestive aspergillosis in a patient with acute myeloid leukaemia.Primary intestinal invasive aspergillosis is rarely reported in leukaemic patients. We describe a case of jejunal invasive aspergillosis in the setting of aplasia following chemotherapy for acute myeloid leukaemia. The diagnosis was confirmed by biopsy obtained during surgery and our polymerase chain reaction (PCR) test confirmed aspergillus flavus as the fungus responsible. This patient had high levels of circulating galactomannan, an antigen secreted by Aspergillus sp., in serum. The ELISA test for galactomannan has been developed to improve the diagnosis of invasive aspergillosis but presents a 5-15% false positive rate. We suggest that some false positive results might be due to non-respiratory invasive aspergillosis, the usual localization of invasive aspergillosis. Our PCR test was also positive in serum. In case of positive results in serum with antigen and/or PCR tests without respiratory symptoms, the intestinal localizations should be investigated.- - - - - - - - - - ranking = 0.66666666666667keywords = antigen (Clic here for more details about this article) |
4/14. arizona hinshawii osteomyelitis with antecedent enteric fever and sepsis. A case report with a review of the literature.A case of arizona osteomyelitis of the spine which occurred 11 months after an episode of gastroenteritis and enteric fever is presented. As close biochemical and antigenic relative of salmonella, arizona infection produces a similar clinical course with gastrointestinal manifestations frequently preceding localized infections by several months. The boney lesion in the present case and in three of the four other cases of arizona osteomyelitis described in the literature was a chronic inflammation which may have a xanthomatous component. The bone destruction caused by arizona infection is less severe than that of tuberculosis or pyogenic osteomyelitis. Proposed treatment of arizona osteomyelitis consists of debridement of the localized infection and long term antimicrobial therapy.- - - - - - - - - - ranking = 0.33333333333333keywords = antigen (Clic here for more details about this article) |
5/14. Primary intestinal aspergillosis after high-dose chemotherapy and autologous stem cell rescue.Primary invasive aspergillosis of the gut is a rare event and is associated with high mortality. We report for the first time on a patient who had isolated aspergillosis of the small bowel after autologous stem cell transplantation. diagnosis of invasive aspergillosis of the gut was based on abdominal pain, galactomannan antigenemia and isolation of aspergillus fumigatus from the stool and was later confirmed by pathohistologic examination. No other site of invasive aspergillosis was evident. The patient was successfully treated with early surgery and combination antifungal therapy.- - - - - - - - - - ranking = 0.33333333333333keywords = antigen (Clic here for more details about this article) |
6/14. cytomegalovirus vasculitis accompanied by an exuberant fibroblastic reaction in the intestine of an AIDS patient.A case of cytomegalovirus (CMV) vasculitis in the intestine of a patient with acquired immune deficiency syndrome (AIDS) is reported. The distal jejunum and ileum had multiple well-demarcated mucosal ulcers and microscopic examination revealed an unusual, exuberant fibroblastic proliferation in the ulcer base. Amidst these fibroblasts, there were several small blood vessels of which the endothelial cells contained cytomegalic inclusion bodies. The lesion showed a Kaposi's sarcoma-like appearance, but spindle cells were negative in immunostaining for factor viii-related antigen or ulex europaeus agglutinin-1. Although the CMV infection was observed in almost all organs, the exuberant fibroblastic proliferation seen in the intestine was not found in other organs. This lesion might represent a peculiar reaction of the immunologically compromised host to the CMV in the intestinal blood vessels.- - - - - - - - - - ranking = 0.33333333333333keywords = antigen (Clic here for more details about this article) |
7/14. Immune dysregulation, polyendocrinopathy, enteropathy, X-linked syndrome (IPEX) associated with pemphigoid nodularis: a case report and review of the literature.The immune dysregulation, polyendocrinopathy, enteropathy, X-linked syndrome (IPEX) is a rare disorder caused by mutations of the FOXP3 gene. The FOXP3 gene encodes a dna-binding protein of the forkhead/winged-helix family and is the central controller of the development of CD4 CD25 regulatory T cells. CD4 CD25 regulatory T cells help prevent autoimmune disease; a deficiency of these cells causes increased immunologic reactivity and autoimmunity. We describe a 14-year-old boy with IPEX syndrome confirmed by mutation analysis of the FOXP3 gene. The patient had chronic dermatitis and later developed bullous pemphigoid. He subsequently formed diffuse prurigo nodularis-like lesions resistant to multiple topical and systemic immunosuppressive medications. These lesions were confirmed by biopsy, direct immunofluorescence, and enzyme-linked immunosorbent assay of the 180 kd bullous pemphigoid antigen to be pemphigoid nodularis. He recently responded to rituximab, allowing discontinuation of his oral prednisone.- - - - - - - - - - ranking = 0.33333333333333keywords = antigen (Clic here for more details about this article) |
8/14. "Occult" mastocytosis with activating c-kit point mutation evolving into systemic mastocytosis associated with plasma cell myeloma and secondary amyloidosis.A case of a 70-year-old man presenting with exsudative enteropathy due to light-chain-associated amyloidosis is reported. The diagnosis of systemic mastocytosis associated with IgG/lambda plasma cell myeloma and secondary generalised amyloidosis was carried out by morphological evaluation of bone marrow biopsy. The c-kit point mutation D816Y was detected by molecular analysis. Two years before, a cystadenolymphoma of the left parotid gland had been removed. A moderate increase of loosely scattered spindle-shaped mast cells, a subpopulation of them expressing CD25, an antigen that is not expressed by normal or reactive mast cells, was shown by retrospective analysis carried out on an intraparotideal lymph node. The c-kit mutation D816Y was shown by the molecular analysis of the lymph node. In summary, the notion that systemic mastocytosis may very rarely be associated with B cell neoplasms and that neoplastic mast cell infiltrates may be obscured because of only a minimal increase of atypical mast cells, which are outnumbered by other non-neoplastic cells in the same tissue, is supported by this case. This finding was preliminarily termed "occult" mastocytosis.- - - - - - - - - - ranking = 0.33333333333333keywords = antigen (Clic here for more details about this article) |
9/14. Autoimmune enteropathy and nephropathy with circulating anti-epithelial cell antibodies.We describe a child with circulating anti-epithelial cell antibodies, autoimmune enteropathy with intestinal villous atrophy, and membranous glomerulonephritis. The patient had persistent diarrhea at 6 months of age, and a small bowel biopsy showed active enteritis, villous atrophy, and crypt hyperplasia. When the patient was, 10 months of age, nephrotic syndrome developed because of membranous glomerulonephritis. Results of tests for circulating immune complexes were negative. Indirect immunofluorescence studies revealed a circulating antibody directed against renal epithelial cells. Circulating antibodies directed against normal small intestine epithelial cells were also detected by the immunoperoxidase technique. Western blot and immunoprecipitation identified a 55-kd antigen, in both small bowel and kidney, that reacted with an antibody in the patient's serum. High-dose prednisone therapy induced a clinical remission, resolution of the small bowel injury, and diminished serum anti-epithelial cell antibodies; after dose reduction, clinical relapse occurred with villous atrophy and reappearance of anti-epithelial cell antibodies. When the patient was 45 months of age, persistent diarrhea recurred despite intravenous administration of corticosteroids, cyclosporine, and total parenteral nutrition. autoantibodies to a 55-kd epithelial cell protein are temporally related to the development of enteropathy and nephropathy. Study of similar patients is needed to determine the role of such antibodies in this disorder.- - - - - - - - - - ranking = 0.33333333333333keywords = antigen (Clic here for more details about this article) |
10/14. Juvenile pernicious anemia associated with intestinal nodular lymphoid hyperplasia and immune deficiency state. Report of a case.A 17-year-old girl with humoral immune deficiency state was observed, in whom pernicious anemia developed one year before her death. The patient died after a very severe diarrheal attack and respiratory tract infection. The necropsy revealed intestinal nodular lymphoid hyperplasia. The immunological evaluation showed depressed IgG and IgM concentration with lack of IgA, however in the patient's blood there were found B lymphocytes containing IgA immunoglobulin. On the effect of crude gastric antigen the patient's lymphocytes showed M.I.F. release, although there were no circulating autoantibodies.- - - - - - - - - - ranking = 0.33333333333333keywords = antigen (Clic here for more details about this article) |
| | Next -> |