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

Eleven patients with Fanconi anemia (FA) underwent bone marrow transplantation (BMT) between March 1985 and May 1990 in a single institution. Ten patients received bone marrow from healthy full human leukocyte antigen (HLA) matched siblings and one patient from her father (one antigen mismatch). Ten patients were conditioned with cyclophosphamide (Cy) at a dose of 5 mg/kg per day for 4 days followed by total body irradiation (TBI) for a total of 600 cGy over 3 days. Six of the 11 patients are alive and have normal reconstitution of their bone marrow. Median follow-up was 72 months (range 42-84). Three of the 10 patients who received Cy and TBI (two HLA compatible, one antigen mismatch) had graft failure. Five patients developed at least grade III acute graft-versus-host disease (GVHD). The rates of graft failure and GVHD are, however, still significantly high. Modification of the conditioning regimen and GVHD prophylaxis is needed to improve the outcome.
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PMID:Bone marrow transplantation in patients with Fanconi anemia: experience with cyclophosphamide and total body irradiation conditioning regimen. 902 15

Allogeneic transplantation is the only form of therapy that enables physicians to cure patients with acute myeloid leukemia (AML) and acute lymphoblastic leukemia (ALL) who do not respond to induction therapy. Thus, patients and family members should be human leukocyte antigen (HLA)-typed soon after the diagnosis to expedite transplantation should induction therapy fail. Transplantation is superior to chemotherapy in patients with AML in second remission. The role of transplantation in ALL other than induction failure is somewhat different in children than in adults. Transplantation appears to be the treatment of choice in children with ALL in second remission regardless of the characteristics of the disease. Adults who relapse off-therapy after prolonged remission should probably be reinduced, whereas those with short remissions probably should go directly to transplant. Transplants from family members incompatible for one antigen result in survival rates similar to those observed with HLA-identical sibling transplants, but transplants from family donors mismatched for two or three antigens have been associated with a substantial increase in graft-versus-host disease (GVHD) and other complications and such transplants should be reserved for patients with few other prospects for cure. Randomized trials are underway to compare peripheral blood stem cells mobilized with granulocyte colony stimulating factor to marrow for H LA-matched transplantation. Results with unrelated donor transplants have improved with time and are approaching those for matched sibling transplants. Early results suggest that umbilical cord blood transplants are feasible with more graft failure but less GVHD than with unmodified marrow.
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PMID:Allogeneic hematopoietic stem cell transplantation for acute leukemia. 904 97

We studied the efficacy and safety of a risk-adapted approach with ganciclovir to prevent cytomegalovirus (CMV) pneumonia in 41 CMV-seropositive recipients of genotypically human leukocyte antigen-identical bone marrow transplants. Prophylaxis with ganciclovir, at a dosage of 2.5 mg/kg twice a day for 14 days, was started when patients were treated with high-dose steroids for acute graft-versus-host disease (i.e., they were considered at high risk for CMV pneumonia), or the drug was given as preemptive therapy when CMV antigenemia developed (i.e., the patients were considered at intermediate risk for CMV pneumonia). Twelve patients (29%) were treated prophylactically and seven patients (17%) preemptively. Only five patients (26%) received a second course of ganciclovir (given preemptively to four patients). Twenty-two patients (54%) never received ganciclovir because they did not fall within these risk groups. None of the 41 patients developed CMV pneumonia. Ganciclovir-related granulocytopenia did not occur (course 1) or was very mild (course 2). We conclude that this approach appears to prevent the development of CMV pneumonia after bone marrow transplantation.
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PMID:A risk-adapted approach with a short course of ganciclovir to prevent cytomegalovirus (CMV) pneumonia in CMV-seropositive recipients of allogeneic bone marrow transplants. 914 90

Even though there is recognized morbidity and death associated with bone marrow transplantation, this procedure has been performed successfully in a substantial number of patients with hemoglobinopathies. However, finding a suitable related donor is often difficult and the morbidity associated with the use of unrelated donors is high. Several reports indicate that fewer than 30% of patients with thalassemia major and fewer than 20% of patients with sickle cell anemia have histocompatible siblings. Human umbilical cord blood (UCB) contains hematopoietic stem cells capable of reconstituting bone marrow. To date, approximately 200 transplantations have been performed with UCBs. Early results suggest that, even with substantial human leukocyte antigen (HLA) incompatibility, a decrease in the incidence of graft-versus-host disease occurs with cord blood. The extent to which HLA incompatibility can be tolerated when cord blood is used has not been determined. These results raise the possibility that UCB obtained from unrelated donors could be used for transplantation in patients with hemoglobinopathies. This review summarizes current data on UCB stem cells used for transplantation in hematologic diseases. The review contains a discussion of the potential uses of UCB for patients with hemoglobinopathies and the value of programs designed to collect UCB from newborn infants with hemoglobinopathies, from siblings of patients with hemoglobinopathies, and from groups of ethnic minorities similar to those in which hemoglobinopathies are found.
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PMID:Umbilical cord blood stem cells: application for the treatment of patients with hemoglobinopathies. 915 73

Children with severe aplastic anemia (SAA) are treated with bone marrow transplantation (BMT) if a human leukocyte antigen (HLA) compatible sibling donor is available, or alternatively with immunosuppressive therapy (IST). Three retrospective trials examining BMT vs IST in pediatric patients treated from 1970-1988 found BMT resulted in a superior survival rate. Advances have been made in general supportive care and in the approach to each of these treatment modalities in the last decade. To compare survival following BMT and IST in a more recent era, we retrospectively analyzed the results of 48 consecutively treated children with SAA presenting to Memorial Sloan-Kettering Cancer Center (MSKCC) between 1983 and 1992. In contrast to the previous studies, the estimated survival of the BMT and IST groups at 120 months are equivalent, 75.6% and 73.8%, respectively. The IST results in our series are superior to the 42-48% (2-10 year) survival previously published, but similar to survival data observed in more recent IST trials employing more intensive immunosuppression (antithymocyte globulin and cyclosporine). The overall BMT survival rates are similar to those previously published, although BMT results improved dramatically during the latter five years of this analysis, with all 11 patients transplanted surviving with a minimum follow-up of 3 years. No surviving BMT patient has extensive chronic graft-versus-host disease (GvHD), a major cause of long-term mortality post-BMT. Therefore, it is likely the BMT survival curve will remain stable. In contrast, the survival curve of the IST patients is likely unstable, since patients are still at risk for relapse or development of clonal disease. Thus, despite overall similar survival rates, we continue to recommend BMT as first-line therapy in pediatric SAA patients with matched sibling donors.
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PMID:Comparison of long-term outcome of children with severe aplastic anemia treated with immunosuppression versus bone marrow transplantation. 920 37

In patients undergoing bone marrow transplantation cryptococcosis is rarely encountered. We report a fatal case of Cryptococcus meningitis in a 12-year-old girl with acute lymphoblastic leukemia (ALL) in second remission who had a transplant from a human leukocyte antigen (HLA)-identical unrelated bone marrow donor. The conditioning regimen was thiotepa, cyclophosphamide, and total body irradiation (TBI); graft-versus-host disease (GVHD) prophylaxis consisted of cyclosporin A, methotrexate, and antilymphocyte globulin (ALG). The patient experienced stage III GVHD responsive to high-dose corticosteroids. On day +54 a thrombotic microangiopathy occurred. On day +64 neurological status worsened; a brain computed tomographic (CT) scan showed hyperdense lesions suggesting fungal infection. Detection of cryptococcal antigen by latex agglutination was positive but India ink stain and culture were negative. Despite treatment with amphotericin B, 5-flucytosine, and granulocyte-macrophage colony-stimulating factor, the patient died 13 days after the diagnosis.
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PMID:Cryptococcal meningitis following a thrombotic microangiopathy in an unrelated donor bone marrow transplant recipient. 926 80

This article describes a gingival squamous cell carcinoma that developed in a 21-year-old woman who received a bone marrow transplant at the age of 16 from her human leukocyte antigen-identical sister as treatment for severe aplastic anemia. Thirty days after transplantation, she presented with cutaneous erythema as a result of acute graft-versus-host disease, and this subsequently evolved into chronic graft-versus-host disease. A lichenoid white plaque of the gingiva developed shortly thereafter, and it began to increase in size rapidly 4 years posttransplantation. Biopsy indicated squamous cell carcinoma arising in this region, apparently associated with chronic graft-versus-host disease. Few reports have described a secondary solid malignancy involving the oral cavity of young adults after bone marrow transplantation.
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PMID:Gingival squamous cell carcinoma in a patient with chronic graft-versus-host disease. 926 19

Every year 2,800 children are diagnosed with leukemia and between 30% and 60% will relapse and need a bone marrow transplant. In addition, children with hematologic, genetic, or immunologic diseases may also require a transplant to be cured. Unfortunately, only 30% of these children will have a human leukocyte antigen-matched sibling donor. The current options for alternative donor sources include matched unrelated donor, mismatched related donor, and unrelated cord blood donor. Compared to a matched sibling donor, each of these options has an increased risk for graft failure and graft-versus-host disease (GVHD). For patients who receive stem cells from matched unrelated donors or mismatched related donors, the risk of graft failure is 5% to 10% and the risk of GVHD approaches 80%. After unrelated cord blood transplants, the graft failure rate is 8%, but this is potentially offset by less severe GVHD. Challenges for nurses include providing anticipatory guidance for patients and families undergoing these novel therapies and devising treatment strategies to manage the complications. Graft failure, GVHD, and infections pose the most significant risks associated with alternative donor transplants.
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PMID:Alternative donor sources in pediatric bone marrow transplantation. 932 95

Bone marrow transplantation has been used for several years in the treatment of hematopoietic system malfunction. However, this particular therapy option has had minimal benefit when the hematopoietic system failure results from radiation exposure, such as that after the Chernobyl accident. In the last ten years, there has been considerable progress in the development of methods to encourage stem cell repopulation with the application of hematopoietic growth factors, and to reconstitute the hematopoietic system with stem cells extracted from the peripheral blood. Problems with allogeneic bone marrow or peripheral blood stem cell transplantation as a therapy option include graft-versus-host disease and a shortage of human leukocyte antigen (HLA)-matched donors. These problems can be overcome if an autologous bone marrow or peripheral blood transplant is performed, but this is not always practical. Another approach to combat these difficulties is the use of umbilical cord blood as a source of donor cells, since placental blood is rich in stem cells and less prone to lead to graft-versus-host disease than mature blood.
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PMID:Potential use of hematopoietic stem cells after radiation injury. 936 17

Marrow transplantation from human leukocyte antigen (HLA) matched related donors offers a high probability of prolonged treatment-free survival for patients with chronic myeloid leukaemia in chronic phase. Delay, patient and donor gender, patient age and previous palliation with busulphan predict outcome in this setting. Because of the median age at diagnosis and the genetics of the HLA system, transplants from HLA-matched related donors are available to less than 15% of newly diagnosed patients. Alternative donors include relatives with minor degrees of incompatibility and HLA-compatible unrelated volunteers. The probability of finding suitable unrelated donors has increased with the development of a network of registries now containing more than 3.6 million donors worldwide. Survival prospects will be improved by transplantation earlier in the course of the disease, better-matched donors and the discovery of new approaches for the prevention of graft-versus-host disease and opportunistic infections.
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PMID:Allografting for chronic myeloid leukaemia. 937 67


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