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)

Peripheral blood lymphocytes from 25 allogeneic bone marrow transplant recipients were studied serially using flow cytometry and two colour analysis. Fourteen patients were transplanted for haematologic malignancies, eight for aplastic anaemia, two for congenital immunodeficiencies and one for Morquio's disease. All patients were alive more than 100 days post-grafting; nine patients had chronic graft-versus-host disease (GVHD). Dual labelling with monoclonal antibodies, CD4/2H4, CD4/4B4, CD8/CD11, CD8/HLA-DR and CD8/Leu 7 was used to analyse the surface phenotypes of lymphocytes. The population of CD4+2H4+ cells was decreased, and CD8+CD11+, CD8+CD11- and CD8+Leu7+ cells were markedly increased in patients with chronic GVHD. The increase of CD8+CD11+, CD8+CD11- and CD8+Leu7+ cells closely correlated with clinical signs of chronic GVHD in each patient. These results suggest that CD8+ cells may play an important role in effector and/or suppressor mechanisms of chronic GVHD and could be used as an indicator of need for and response to treatment.
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PMID:Increased numbers of CD8+CD11+, CD8+CD11- and CD8+Leu7+ cells in patients with chronic graft-versus-host disease after allogeneic bone marrow transplantation. 169 37

Two patients treated by unrelated bone marrow transplantation were reported. Case 1 was an eight-year-old boy with Morquio's disease received bone marrow graft from an HLA one-locus mismatched, MLC non reactive unrelated donor. The patient was prepared with conventional dose of cyclophosphamide, and thoracoabdominal irradiation. For the prophylaxis of GVHD, three drug regimen consisted of methotrexate (MTX), cyclosporine A (CsA), and prednisolone (PSL) was administered. Engraftment was prompt, and grade I of acute GVHD developed, which resolved with increased dose of PSL. Case 2 was a ten-month-old boy with juvenile chronic myelogenous leukemia received bone marrow graft from an HLA fully matched, MLC non reactive unrelated donor. Preconditioning regimen consisted of total body irradiation, VP-16, and cytosine arabinoside. MTX, CsA, and methylprednisolone were administered to prevent GVHD, but grade II of acute GVHD developed, which resolved with prolonged course of PSL. Both cases are alive and well, without chronic GVHD. In conclusion, unrelated donor bone marrow transplantation may be a useful to treat hematologic malignancies, aplastic anemia, and some inherited diseases.
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PMID:[Result of allogeneic bone marrow transplantation from unrelated donor]. 239 12

Morquio A syndrome features systemic skeletal dysplasia. To date, there has been no curative therapy for this skeletal dysplasia. No systemic report on a long-term effect of hematopoietic stem cell transplantation (HSCT) for Morquio A has been described. We conducted HSCT for 4 cases with Morquio A (age at HSCT: 4-15years, mean 10.5years) and followed them at least 10years (range 11-28years; mean 19years). Current age ranged between 25 and 36years of age (mean 29.5years). All cases had a successful full engraftment of allogeneic bone marrow transplantation without serious GVHD. Transplanted bone marrow derived from HLA-identical siblings (three cases) or HLA-identical unrelated donor. The levels of the enzyme activity in the recipient's lymphocytes reached the levels of donors' enzyme activities within two years after HSCT. For the successive over 10years post-BMT, GALNS activity in lymphocytes was maintained at the same level as the donors. Except one case who had osteotomy in both legs one year later post BMT, other three cases had no orthopedic surgical intervention. All cases remained ambulatory, and three of them could walk over 400m. Activity of daily living (ADL) in patients with HSCT was better than untreated patients. The patient who underwent HSCT at four years of age showed the best ADL score. In conclusion, the long-term study of HSCT has demonstrated therapeutic effect in amelioration of progression of the disease in respiratory function, ADL, and biochemical findings, suggesting that HSCT is a therapeutic option for patients with Morquio A.
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PMID:Hematopoietic stem cell transplantation for Morquio A syndrome. 2645 13