Gene/Protein
Disease
Symptom
Drug
Enzyme
Compound
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Gene/Protein
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Target Concepts:
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Query: EC:2.1.1.69 (
BMT
)
2,655
document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)
Patients who received bone marrow transplantation (=
BMT
) for the treatment of severe combined immunodeficiency (= SCID), and who were reported in the medical literature from 1968 to 1977, were collected and analysed. Eighteen of these 80 children are still alive, 10 months to 9 years after transplantation. It is thus the first successful form of therapy for this otherwise invariably fatal disease. Fifteen of the 18 survivors received bone marrow cells from
HLA
and MLC compatible donors; the remaining 3 survivors received grafts from MLC-compatible but
HLA
-incompatible donors. Bone marrow transplantation is the treatment of choice for SCID when recipient and donor are
HLA
- and MLC-identical. All patients who received MLC-incompatible grafts died, and bone marrow transplantation for SCID from MLC-incompatible donors should be abandoned. Milt-to-severe graft-versus-host disease (= GVHD) occurred in spite of
HLA
- and/or MLC-compatibility, with some correlation to the number of cells transplanted. This should preferably be kept below 50 million cells per kilo body weight. Infection was the chief cause of death in all groups. Strict reverse isolation, bowel decontamination and routine pre- and post-transplant Pneumocystis carinii prophylactic treatment are recommended. The clinical picture and laboratory findings of these 80 children before
BMT
did not differ from non-transplanted SCID patients. Three of the 18 survivors are adenosinedeaminase deficient.
...
PMID:Bone marrow transplantation for severe combined immunodeficiency disease. Reported from 1968 to 1977. 3 63
Seven children underwent
BMT
for acute megakaryoblastic leukemia (AMKL). They were assessed for clinical, hematologic, and cytogenetic findings as well as response to treatment. The diagnosis of AMKL was established by cytochemistry, immunophenotyping and/or platelet-peroxidase reactivity. Patients had received various prior chemotherapies. One was in first remission, another in second remission and five were in relapse at the time of admission for transplant. Marrow donors included an
HLA
identical sibling (one), phenotypically
HLA
identical unrelated (two) and partially
HLA
identical family members (four). Five patients achieved engraftment, one rejected the graft and died on day 20 after a second unrelated transplant and one died from infection on day 5. Two patients relapsed within the first month after transplant and died of recurrent leukemia. Another died of a second malignancy on day 2232. Two patients survive disease-free more than 3.8 and 4.3 years after transplant.
...
PMID:Acute megakaryoblastic leukemia in children: treatment with bone marrow transplantation. 146 99
In order to determine the incidence and causes of death during the first 100 days after
BMT
(early deaths) in a pediatric population we have examined data reported in the AIEOP
BMT
Registry. Up to July 1990, data on 486 children who underwent allogeneic (180) or autologous (306)
BMT
were evaluable. The children had acute lymphoblastic leukemia (148 cases), acute non-lymphoblastic leukemia (127 cases), neuroblastoma (82 cases), chronic myelogenous leukemia (15 cases), aplastic anemia (nine cases), solid tumors, lymphoma, immunodeficiency or storage diseases. The overall survival is 55% for allogeneic
HLA
matched and 38% for autologous transplants at 5 years, 24% for
HLA
mismatched graft at 2 years. Out of the 486 children, 70 (14%) died during the first 100 days after
BMT
: 33/306 (11%) after autologous
BMT
, 24/150 (16%) after allogeneic matched
BMT
and 13/30 (43%) after mismatched
BMT
. Causes of early death were as follows: disease progression: 12 children (10/306 after autologous and 2/180 after allogeneic
BMT
); infection: 12 children (five after autologous and seven after allogeneic
BMT
); interstitial pneumonitis: 21 children (seven after autologous and 14 after allogeneic
BMT
); cardiac failure: five children (four after autologous
BMT
); veno-occlusive disease: eight children (three after autologous, five after allogeneic
BMT
); acute renal failure: three children (one after autologous and two after allogeneic
BMT
); multiple organ failure: two cases (one after autologous
BMT
); cerebral hemorrhage: three children (one after autologous
BMT
); hypertension: one child; acute GVHD: three children (12% of early deaths after allogeneic
BMT
).
...
PMID:Early deaths in children after BMT. Bone Marrow Transplantation Group of the Italian Association for Pediatric Hematology and Oncology (AIEOP) and Gruppo Italiano Trapianto di Midollo Osseo (GITMO). 146 3
A 21-year-old man who had an
HLA
-identical sibling donor
BMT
for chronic myeloid leukaemia developed grade IV acute GVHD of the liver that was unresponsive to corticosteroids and anti-IL2 receptor monoclonal antibody. He was treated with an orthotopic liver transplant and is currently well 6 months later with normal liver function and no evidence of GVHD in the transplanted liver.
...
PMID:Orthotopic liver transplantation for hepatic GVHD following allogeneic BMT for chronic myeloid leukaemia. 146 11
The first case of allogeneic bone marrow transplantation in acute myelogenous leukemia (AML) done in Mexico is reported. The patient was a 26 year old Mexican woman who in October 1987 was diagnosed of having AML of the M2 subtype. After three cycles of the TADOP regimen (6-thioguanine, cytosine-arabinoside, doxorubicin, vincristine & prednisone), the patient entered complete remission. Unfortunately, after a seven month period of remission she suffered a relapse which was refractory to a new chemotherapy cycle. On 9/14/88 an allogeneic
BMT
from her
HLA
identical brother was performed. The conditioning regimen consisted of busulfan and cyclophosphamide. Prophylaxis for GVHD consisted of cyclosporine and methylprednisolone. The posttransplantation course was satisfactory, reaching > 500 neutrophils x 10(9)/L on day 14 and > 50,000 platelets x 10(9)/L without support on day 23 posttransplant. The patient developed fever of unknown etiology, which was satisfactorily resolved with ceftazidime, vancomycin and metronidazole. She also presented a grade II oral and esophageal mucositis. As a late complication, on day 90 posttransplant, she developed a bilateral pneumonia which was resolved with sulfamethoxazole-trimethoprim administration. Up to the time of this report (40 months posttransplant) the patient is completely asymptomatic, is under no immunosuppression, and shows no evidence of graft versus host disease or recurrent leukemia.
...
PMID:[Bone marrow transplantation in Mexico. Report of the 1st successful case in acute myeloblastic leukemia. Grupo de Trasplante Medular Oseo del INNSZ]. 148 82
This report describes the qualitative analysis of T cell receptor (TCR) repertoire regeneration in recipients of
BMT
. RNA samples from patient and control peripheral blood lymphocytes were prepared and tested for the presence of multiple V alpha and V beta transcripts by the polymerase chain reaction. TCR V gene expression was highly diverse within the first 6 months post-transplantation in recipients receiving either T cell-depleted or T cell-replete marrow, and in
HLA
mismatched as well as matched donor-recipient pairs. The sequencing of TCR message from
BMT
recipients also demonstrated J gene diversity and apparently normal junctional diversity at the V-J alpha join. Thus, T cell pools in
BMT
recipients are largely heterogeneous, not mono- or oligoclonal.
...
PMID:Reconstitution of the T cell receptor alpha beta repertoire in recipients of allogeneic BMT. 149 Feb 2
We describe here a 3-year-old boy with juvenile chronic myelomonocytic leukemia and monosomy 7 who underwent a second
HLA
matched bone marrow transplant from his sister. He developed hypothyroidism due to acute suppurative thyroiditis in the recovery phase of
BMT
. This is an extremely rare complication of
BMT
.
...
PMID:Acute suppurative thyroiditis complicating second allogeneic transplant for juvenile CMML. 149 Feb 4
Skin tissue of a healthy chimera 7 years after
HLA
identical bone marrow transplantation was found to express a minor histocompatibility (mH) antigen against which cytotoxic T lymphocytes (CTLs) had been discovered at a time of acute graft-versus-host disease (aGVHD). We were prompted to investigate the apparent tolerance to this persistent mH antigen and used limiting dilution analysis to monitor in vitro anti-host CTL responses in time after bone marrow transplantation. A high anti-host CTL precursor frequency was found during acute GVHD, declining in time until beyond detection level in the healthy chimera 7 years after transplantation. In this case report (i) CTL precursor frequencies are used for the first time to monitor in vitro tolerance induction to persistent host mH antigens after
HLA
identical
BMT
in man; and (ii) it is shown that LDA may be a potential tool for quantification and specificity analysis of CTL responses to mH antigens.
...
PMID:Acquired tolerance for minor histocompatibility antigens after HLA identical bone marrow transplantation. 154 May 48
A total of 21 multiply transfused patients with severe aplastic anemia (SAA) were treated with bone marrow transplantation between March 1985 and September 1990: 20 allogeneic and one syngeneic transplants. A positive response in mixed lymphocyte culture (MLC) was also noted in 7 allogeneic recipients. Pregraft conditioning included high-dose cyclophosphamide (CY) 200 mg/kg over 4 consecutive days, followed by 300 cGy total-body irradiation the day before
BMT
. Seventeen patients older than 14 years received additional donor buffy-coat cells infusion for 5 days posttransplant. A combination of methotrexate and cyclosporine was used for prophylaxis of graft-versus-host disease. Seventeen patients were alive with a functional graft, and Kaplan-Meier product limit estimates showed a 80.95% probability of survival at 67.7 months. There were 4 deaths: two died of primary graft failure, one from secondary rejection, and the other from chronic GVHD-related complications. Acute GVHD, grade I was noted in only one patient (5.6%). In contrast, chronic GVHD was observed in 10 out of 18 (55.6%) evaluable patients. Venoocclusive liver disease and interstitial pneumonitis were not diagnosed. Our findings indicate that the combination of CY/TBI/BC is well tolerated and results in a low incidence of graft failure/rejection in multiply transfused Chinese patients who received transplants for SAA. The MTX/CsA combination was confirmed as being remarkable in reducing the incidence and severity of acute GVHD. For patients with SAA under the age of 40, with an
HLA
-identical sibling, we highly recommend
BMT
as the treatment of choice.
...
PMID:Bone marrow transplantation for severe aplastic anemia--a study of twenty-one Chinese patients in Taiwan. 154 48
BMT
is an effective treatment for certain malignant and nonmalignant conditions. The source of the marrow is autologous or allogeneic. An allogeneic donor can be an
HLA
-matched related or unrelated donor. The patient undergoes intense chemoradiotherapy to remove remaining malignant cells and obliterate the immune system, thus allowing growth of the new bone marrow cells. Complications of conditioning therapy include pancytopenia and distinct organ toxicities. Astute nursing care is critical in managing the care of
BMT
patients. Assessment and numerous, interrelated interventions are required. Late complications of
BMT
relate to the conditioning therapy and to the transplant itself. As
BMT
becomes more readily available as a treatment, economic issues related to the cost of care and the allocation of resources challenge health care providers.
...
PMID:Bone marrow transplantation. 156 4
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