1/52. A case of McLeod syndrome with chronic renal failure.A 50-year-old man with the rare McLeod syndrome, associated with glomerular lesion to the end stage of chronic renal failure and death, is reported. McLeod syndrome is an X-linked recessive disorder on the basis of abnormal expression of the Kell blood group antigens and absence of erythrocyte surface Kx antigen. Most often the clinical and pathological findings are retinitis pigmentosa to blindness, progressive chronic neuropathy, cortical atrophy, dilated cardiomyopathy, and glomerular lesion with chronic renal failure. Among the laboratory parameters the most important are very low level of cholesterol and triglycerides, then various numbers of acanthocytes in peripheral blood smears and sometimes in urine (as in our case).- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
2/52. Combined histocompatibility leukocyte antigen-matched donor bone marrow and renal transplantation for multiple myeloma with end stage renal disease: the induction of allograft tolerance through mixed lymphohematopoietic chimerism.BACKGROUND: Experimental and clinical evidence has demonstrated that the establishment of allogeneic chimerism after bone marrow transplantation may provide donor-specific tolerance for solid organ allografts. methods: Based on the preliminary results of a clinical trial using nonmyeloablative preparative therapy for the induction of mixed lymphohematopoietic chimerism, we treated a 55-year-old woman with end stage renal disease secondary to multiple myeloma with a combined histocompatibility leukocyte antigen-matched bone marrow and renal transplant after conditioning with cyclophosphamide, antithymocyte globulin, and thymic irradiation. RESULTS: The posttransplant course was notable for early normalization of renal function, the absence of acute graft-versus-host disease, and the establishment of mixed lymphohematopoietic chimerism. cyclosporine, which was the only posttransplant immunosuppressive therapy, was tapered and discontinued on day 73 posttransplant. No rejection episodes occurred, and renal function remains normal on day 170 posttransplant (14 weeks after discontinuing cyclosporine). Although there is presently no evidence of donor hematopoiesis, there is evidence of an ongoing antitumor response with a recent staging evaluation showing no measurable urine kappa light chains. The patient remains clinically well and is off all immunosuppressive therapy. CONCLUSION: This is the first report of the deliberate induction of mixed lymphohematopoietic chimerism after a nonmyeloablative preparative regimen to treat a hematological malignancy and to provide allotolerance for a solid organ transplant.- - - - - - - - - - ranking = 2.5keywords = antigen (Clic here for more details about this article) |
3/52. A case of late onset cyclosporine-induced hemolytic uremic syndrome resulting in renal graft loss.A case of late onset hemolytic uremic syndrome (HUS) associated with cyclosporine (CYA) is described in this report. A 50-yr-old man with end-stage renal failure due to immunoglobulin a (IgA) nephropathy received a renal transplant from his wife. Human leucocyte antigen was completely unmatched. Immunosuppressant was a combination of prednisolone, azathioprine, and CYA. He was discharged 1 month after transplantation, with no episode of acute rejection. Twenty-one months after transplantation, his platelet count and hematocrit began to decrease and lactate dehydrogenase began to increase. Graft biopsy showed thrombotic microangiopathy and recurrent IgA nephropathy. Graft function was rapidly deteriorated and methylprednisolone pulse therapy was not effective. Twenty-five months after transplantation, he returned to a regular hemodialysis. hemolysis was immediately improved after a reduction of the dose of CYA to 50 mg/d. The trough level of CYA was less than 200 ng/mL in most periods of his clinical course. blood pressure was high throughout the clinical course. Although acute vascular rejection or malignant hypertension could also cause a thrombotic microangiopathy, CYA was most likely a cause of HUS in the present case because of the following reasons: neither anti-acute rejection therapy nor an adequate control of his blood pressure was effective in improving clinical features of HUS; hemolysis and thrombocytopenia disappeared immediately after the reduction of the dose of CYA to 50 mg/d. It has been reported that HUS carried poor prognosis only when occurring shortly after transplantation in cadaver kidney recipients. The present transplant was from a living donor and HUS occurred 21 months after transplantation and was severe enough to result in graft loss. High blood pressure might be one of the predisposing factors of HUS associated with CYA in the present case. CYA should be stopped and other alternative immunosuppressants should be given in cases of acute graft deterioration with hemolysis and thrombocytopenia, irrespective of the interval from transplantation, CYA dose, or CYA trough level.- - - - - - - - - - ranking = 0.5keywords = antigen (Clic here for more details about this article) |
4/52. Evidence of recurrent type I diabetes following HLA-mismatched pancreas transplantation.Type 1 diabetes mellitus is considered as an autoimmune disease against beta cells. Diabetes recurrence after pancreas transplantation is well known in HLA-identical twins while it is rarely reported in recipients of cadaveric pancreatic grafts. In the present case report, diabetes recurrence occurred in a recipient who underwent cadaveric combined pancreas kidney transplantation. Seven years after transplantation the patient exhibited progressive hyperglycemia needing insulin therapy while the renal graft was well functioning. The diagnosis of recurrent disease was obtained on the histological features such as selective loss of beta cells without clear signs of insulitis and on the presence of markers (GAD 65 and IA-2) for humoral autoimmunity. It is intriguing that, at the time of recurrence of type 1 diabetes, the patient had stopped steroids and azathioprine, while only cyclosporine was maintained as immunosuppressive treatment. Our case report underlines the relevance of studying the humoral autoimmune response directed to islet autoantigens in cadaveric pancreas allograft recipients. Furthermore, it suggests that an efficient immunosuppressive treatment after transplantation may be able to reduce the autoimmune response against the pancreatic allograft.- - - - - - - - - - ranking = 0.5keywords = antigen (Clic here for more details about this article) |
5/52. hemoperitoneum due to acute cytomegalovirus infection in a patient receiving peritoneal dialysis.A 27-year-old man receiving continuous ambulatory peritoneal dialysis (CAPD) developed high-grade fever, dyspnea, and hemoperitoneum 32 months after the start of CAPD. A chest computed tomograph showed fine reticular shadows in the bilateral lower lung fields. cytomegalovirus (CMV) antigenemia were detected, and immunoglobulin (Ig) M and IgG antibodies for CMV were also positive. The absolute counts of helper T cells (478/microL) and the ratio of helper T cells/suppressor T cells (0.25) decreased, despite no evidence of hematologic or immunologic diseases, including human immunodeficiency virus (hiv) or human T cell lymphoma virus-1 (HTLV-1) infection, or the use of immunosuppressive drugs. All symptoms, including hemoperitoneum and the ratio of helper T cells/suppressor T cells, improved gradually and spontaneously. Acute and primary cytomegalovirus (CMV) infection induced hemoperitoneum in a patient who was receiving CAPD.- - - - - - - - - - ranking = 0.5keywords = antigen (Clic here for more details about this article) |
6/52. hepatitis b virus infection and the response to erythropoietin in end-stage renal disease.In patients with end-stage renal disease (ESRD), viral or bacterial infections are postulated to abolish or impair response to recombinant erythropoietin (Epogen). However, previous reports revealed that response to Epogen among hemodialysis patients with a particular viral infection--human immunodeficiency virus (hiv)--seems to be variable and is independent of illness severity. To further explore the issue of response to Epogen in hemodialysis patients with viral infection, we retrospectively studied four patients with hepatitis b virus infection over a 3 month period to compare their response to Epogen and endogenous erythropoietin levels with those of a control group of patients without hepatitis b virus infection. Weekly predialysis hematocrit, and monthly serum albumin concentration, transferrin saturation as well as percent reduction of urea were obtained from patient records, and mean values were calculated for each subject. Mean age of the patients (n = 4) was 63 /- 7.5 years compared with 55 /- 23 years for the control subjects (n = 4)(p = 0.02). The mean hematocrit of the study patients was 33.7 /- 2.8% compared with 34.7 /- 4.9% in the control subjects (p = 0.49), and the mean endogenous erythropoietin level in the study patients was 27 /- 22 mlU/ml compared with 5.7 /- 1.9 mlU/ml in the control group (p = 0.001). The mean dose of thrice weekly Epogen, both at onset of the study and when endogenous erythropoietin was measured, was 61 /- 19 U/kg body weight in the patients, compared with 74 /- 8 U/kg body weight in the control subjects (p = 0.002). We conclude that patients with ESRD and hepatitis B surface antigenemia respond to Epogen as well as their counterparts without hepatitis b virus infection. In addition, patients with hepatitis B surface antigenemia have much higher serum levels of endogenous erythropoietin and require less exogenous erythropoietin injections than their counterparts.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
7/52. Heterotopic ovarian splenoma: report of a first case.We present a case of ovarian splenoma, a form of heterotopic splenic hamartoma consisting of red pulp tissue. The hamartoma was located in ovarian stroma in an otherwise normal ovary. The histology showed interanastomosing vascular channels of splenic sinusoidal red pulp lined by cells that were immunoreactive for antibodies to von Willebrand antigen and CD8, findings consistent with splenic lining cells. The sinuses were lined by cuboidal to flattened cells with ovoid and grooved bland-looking nuclei. Ultrastructurally, the tumor cells showed weibel-palade bodies and lysosomes. To our knowledge, this is the first case of splenic tissue arising in an ovary, and it underlines the trend noted in the literature that splenic hamartoma,although a rare entity, can arise in many retroperitoneal organs, including the ovary.- - - - - - - - - - ranking = 0.5keywords = antigen (Clic here for more details about this article) |
8/52. HBV-related cutaneous periarteritis nodosa in a patient 16 years after renal transplantation: efficacy of lamivudine.Cutaneous periarteritis nodosa (PAN) is a clinical feature characterized by chronic, benign course; its pathogenesis is unknown. In patients submitted to renal transplantation cutaneous PAN is a rare complication. We report a case of cutaneous PAN associated with the reappearance of hepatitis B antigen 16 years after kidney transplantation. A 44-year-old man underwent successful renal transplantation in June 1980. In December 1996 he presented multiple painful erythematous subcutaneous nodules on both legs. skin lesion biopsy showed the presence of cutaneous PAN. Six months later laboratory data demonstrated the presence of HbsAg. HBeAg, HBcAb and detectable HBV-dna serum by polymerase-chain-reaction (PCR) assay. Anti-HBs and anti-HBe proved negative. In July 1998 the laboratory tests showed an important increase of HBV-dna (5.1 billion by Branched dna), and so lamivudine (100 mg/day) was introduced. HBV-dna became undetectable by PCR after 3 months of therapy. Seven months later a new skin biopsy was performed. The typical signs of PAN were no longer evident. As HBV infecion was demonstrated six months after the clinical appearance of the PAN, in a patient who was believed to be immune to the virus, it is possible that, in the early stages, the hepatitis B antigen title was methodologically indeterminable, but sufficient to form circulating immune complexes responsible for vasculitis primer. lamivudine therapy resulted efficacious in favouring the regression of cutaneous PAN, but its long-term efficacy requires further evaluation as regards potential selection of drug resistant hepatitis b virus (HBV) mutants during treatment.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
9/52. BK polyomavirus interstitial nephritis in a renal transplant patient with no previous acute rejection episodes.A renal transplant patient treated with tacrolimus and mycophenolate-mofetil (MMF) developed progressive graft function deterioration 10 months after transplantation. biopsy of the graft showed severe, focally accentuated interstitial inflammation with focal tubulitis and tubular necrosis, and medium-severe interstitial fibrosis with focal tubular atrophy. Glomerular and vascular structures were preserved. On careful examination, in some sections, tubular epithelial cells showed a definite increase with deformation of the nuclear shape, chromatin irregularities with peripheral dislocation and inclusion bodies. These cytopathic changes suggested polyoma virus infection ("decoy cells"). Subsequent screening of the urinary sediment confirmed the presence of many "decoy cells". Immunohistochemical analysis of the biopsy showed many tubular cells were strongly positive for the SV 40 antigen, specific for BK polyoma virus. A diagnosis of interstitial nephritis due to BK polyoma virus was made, though the coexistence of cellular rejection could not be excluded. At variance with previous reports, our patient had not had repeated episodes of rejection before biopsy or heavy immunosuppressive treatment, such as ALG, OKT3, after transplantation. This case shows that even in the absence of vigorous anti-rejection therapy an immunosuppressive regimen based on tacrolimus and MMF may involve the risk of BK polyoma virus- associated interstitial nephritis.- - - - - - - - - - ranking = 0.5keywords = antigen (Clic here for more details about this article) |
10/52. Laboratory investigations following an unexpectedly positive crossmatch result in a patient awaiting renal transplantation.In the preparation of patients for renal transplantation tests of human leucocyte antigen (HLA) sensitisation are performed to detect "unacceptable" hla antigens that, if present on donor cells, would be expected to result in a positive crossmatch. Individuals bearing such specificities may then be excluded from consideration as donors. Unexpected positive crossmatch results are sometimes obtained when a serum specificity has not been detected on screening. Failure to identify a donor relevant HLA antibody in a recipient at the time of crossmatch may result in hyperacute rejection of the graft. This report describes laboratory investigations performed after a positive crossmatch result in a live donor situation. The pattern of crossmatch results indicated that reactivity resulted from HLA class I antibody. Previously performed serum screening using a standard complement dependent cytotoxicity technique had failed to identify donor relevant antibody specificities in the recipient. Retrospective flow cytometric screening of the same serum samples identified an HLA-A24 specificity of donor relevance. The lower sensitivity of methods used for routine serum screening compared with those used for crossmatching accounts for the findings in this case. The laboratory has amended its serum screening protocol to include flow cytometric analysis.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
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