Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0018133 (graft-versus-host disease)
 18,032 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 13-yr-old boy was diagnosed as T cell lymphoma. After the second remission, he underwent BMT from an HLA-identical, MLC negative sibling donor. After BMT, he developed grade II acute GVHD. GVHD was improved by pulsed steroid therapy using prednisolone. About 12 months after BMT, he developed bronchiolitis obliterans, sicca syndrome, and leukoderma, which were related to chronic GVHD. Pulsed steroid therapy was carried out twice, and his condition improved. Twenty-seven months after BMT, he developed nephrotic syndrome. A renal biopsy was performed, and the diagnosis was histologically membranous nephropathy and focal glomerular sclerosis. The response to steroids was not satisfactory. After 5 weeks, dipyridamole was added, but proteinuria persisted. Proteinuria disappeared 8 weeks after the addition of cyclosporine. The second biopsy after 5 months of treatment revealed an improvement in the renal lesions. The patient showed a low T4 to T8 ratio of T-lymphocytes at the onset of nephrotic syndrome. However after treatment with cyclosporine, the ratio gradually increased. These findings suggested the nephrotic syndrome in this patient was related to renal involvement in the course of chronic GVHD.
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PMID:[Nephrotic syndrome related to chronic graft versus host disease after allogeneic bone marrow transplantation in a patient with malignant lymphoma]. 899 26

Face vascularized composite allografts (FVCAs) have helped patients with severe facial disfigurement, with acute rejection now largely controlled through iatrogenic immunosuppression. However, little is known regarding the incidence and mechanism(s) of more long-term pathologic alterations in FVCAs that may affect function and graft durability. Protocol surveillance biopsy specimens for up to an 8-year interval in 7 patients who received FVCAs at our institution revealed histopathologic evidence of chronic rejection. Clinical manifestations included features of premature aging, mottled leukoderma accentuating suture lines, telangiectasia, and dryness of nasal mucosa. Pathologic changes consisted of epidermal thinning accompanied by discrete foci of lymphocyte-mediated cytotoxicity, hyperkeratosis, follicular plugging, vascular ectasia, and sclerosis beneath the epidermal layer associated with collagen type I deposition. Genomic interrogation and immunohistochemistry of sclerotic zones revealed upregulation of the AP-1 pathway components, JunB and c-Fos, previously implicated in overproduction of type I dermal collagen in the setting of systemic sclerosis. We conclude that some patients develop chronic rejection in FVCAs with striking similarities to alterations seen in certain autoimmune cutaneous disorders (lupus erythematosus and scleroderma/chronic sclerodermoid graft-versus-host disease). Identification of relevant pathways and genes, such as JunB and c-Fos, may provide new targets for preventative therapies for chronic immune-mediated changes in vascularized composite allografts.
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PMID:Chronic rejection of human face allografts. 3031 35