Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts: Gene/Protein Disease Symptom Drug Enzyme Compound   Target Concepts: Gene/Protein Disease Symptom Drug Enzyme Compound

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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)

Two patients with nasopharyngeal carcinoma (NPC) were treated with "marrow-lethal" chemoradiotherapy and infusion of cryopreserved autologous marrow. Patient AUT 04 (unique patient number), a 28-year-old male, was referred to us because of the right cervical lymphadenopathy. He was diagnosed NPC at stage II (T1N2M0). A trial of combined-modality therapy using autologous bone marrow transplantation (auto-BMT) was scheduled for possible cure. Before auto-BMT, he was treated with a conventional dose of radiation to the nasopharyngeal and bilateral cervical areas. For the pretransplant marrow-lethal therapy, he received high-dose cyclophosphamide (CY), 120mg/kg, and 1,000 rad total body irradiation (TBI). Within 24 hr after TBI, previously cryopreserved-thawed autologous bone marrow cells (1.37 X 10(8)/kg) was infused to the patient. Rapid and complete hematologic recovery was observed and he is now alive in a disease-free remission state 28 months after transplantation. Patient AUT 08, a 18-year-old female, was referred to us for the treatment of head and neck tumor with auto-BMT. She was diagnosed NPC at stage IV (T4N2M0). After cryopreservation of bone marrow, she was treated with local radiation and doxorubicin (80mg X 2d). Then she was conditioned with high-dose CY and upper half body irradiation and received auto-BMT (marrow dose: 1.2 X 10(7)/kg). Successful engraftment was obtained with tumor response but she developed recurrence of the disease 12 months after transplantation. Although the data are too preliminary, auto-BMT is evaluated as one of the favorable treatment approaches for some selected patients with NPC.
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PMID:[Autologous bone marrow transplantation in the treatment of nasopharyngeal carcinoma--report of 2 cases]. 634 32

We report on a 16 year old girl with relapsed Ki-1 lymphoma and a very poor prognosis. The initial manifestation was multiple bone metastases and lymphadenopathy. The patient achieved remission with modified adriamycin, bleomycin, vincristine, daunomycine therapy. However, 14 months after the completion of therapy, relapse occurred in a new cervical lymph node on the left side. After preparation with chemotherapy and total lymphoid irradiation (TLI) the patient underwent autologous bone marrow transplantation (A-BMT). Ki-1 lymphoma shows clinically diverse symptoms, but hematopoietic stem cell transplantation should be performed in relapsed cases. It may be effective to give TLI followed by A-BMT for patients such as ours who have lymph node involvement without bone marrow metastasis.
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PMID:Successful autologous bone marrow transplant for relapsed Ki-1 lymphoma. 757 63

This report describes a child with a severe phenotype of autoimmune lymphoproliferative syndrome (ALPS) who developed progressive disease requiring stem cell transplantation. This severe form of ALPS was associated with a novel Fas gene splice site mutation that resulted in functional deletion of exons 8 and 9. While this child shared many clinical features with previously described ALPS cases, including massive lymphadenopathy and circulating alphabeta+ CD3+CD4-CD8-T cells, his disease progressed despite immunosuppressive therapy to a clinically aggressive oligoclonal lymphoproliferation which resembled a diffuse large cell non-Hodgkin's lymphoma. After partial remission was achieved with cytotoxic therapy the patient underwent BMT from an unrelated donor. This is the first reported case of ALPS in which BMT was successfully attempted for correction of a Fas deficiency.
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PMID:Correction of autoimmune lymphoproliferative syndrome by bone marrow transplantation. 972 73

Between June 1988 and May 1996, 428 patients underwent allogeneic BMT (288 related donor (RD) and 140 unrelated donor (UD)) at the Vancouver General Hospital. Eight patients (UD six and RD two) developed a post-transplant lymphoproliferative disorder (PTLD). Median age at BMT was 38 years (range 22-51). Five of the six UD allografts were T cell depleted. Cyclosporine+/-methotrexate was used for GVHD prophylaxis. All eight patients developed GVHD; in six this was refractory to treatment with corticosteroids. Rabbit antithymocyte globulin (ATG) or an anti-CD5-ricin A chain immunotoxin (Xomazyme) was used as second-line therapy for GVHD. Presentation with PTLD occurred at median day 90.5 (range 34-282) post BMT. Five of the eight patients had a rapidly progressive course characterized by fever, lymphadenopathy, lung and liver involvement and died within 3-8 days. PTLD was an incidental finding at post mortem examination in two patients. The remaining patient had localized disease and recovered. Pathological analysis revealed two morphological patterns; diffuse large B cell lymphoma (DLBC lymphoma, five patients) and polymorphous B cell hyperplasia (PBCH, three patients). EBV expression was positive in all eight cases and monoclonality was demonstrated in seven cases. In multivariate analysis, T cell depletion of the allograft (P=0.0001, relative risk (RR)=30.5), anti-T cell therapy for GVHD (P=0.006, RR=12.7) and acute GVHD grades 3-4 (P=0.04, RR=7.7) were the significant factors for development of PTLD. In conclusion, we have identified two forms of PTLD after BMT: one is characterized by disseminated disease with a rapidly progressive and often fulminant course and the other by localized, relatively indolent disease. Morphology, EBV positivity and clonality do not appear to correlate with the clinical course. The major risk factors for development of PTLD after BMT are ex vivo T cell depletion of the allograft and in vivo anti-T cell therapy for GVHD.
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PMID:Lymphoproliferative disorders following allogeneic bone marrow transplantation: the Vancouver experience. 984 95

Translocation t(9;22) or Philadelphia chromosome (Ph)/BCR-ABL rearrangement positive acute lymphoblastic leukemia (Ph/BCR+ ALL) is associated with a very short survival of about one year in most patients. We analyzed long-term outcome of 76 adults with Ph/BCR+ ALL, in order to detect which factors were associated with longer survival. Modifiable prognostic factors included type of treatment, allogeneic marrow transplant (allo-BMT), and early anthracycline dose intensity (high = H/A, low = L/A); unmodifiable factors were age, gender, FAB morphology, phenotype, blast count, P190/210 transcript, hepatospleno-lymphadenopathy, LDH level. Median patient age was 43 years (range 15-71). Four favorable prognostic factors (FPF) were found associated with greater likelihood of complete remission (blast count < 50 x 10(9)/l, p = 0.08), longer remission duration (age < 50 years, p < 0.001; H/A, p < 0.05), and lower relapse rate (allo-BMT, p = 0.017). Age and anthracycline dose intensity exerted a synergistic prognostic effect. According to the cumulative incidence of FPF in each patient (FPF 0-1 = 29, 2-3 = 42, 4 = 5), the probability of survival increased from nil to 0.22 to 0.60 at 5 years (p < 0.005). Adult Ph/BCR+ ALL is relatively sensitive to anthracyclines, which therefore should be prescribed at full dosage to patients not eligible to allo-BMT or in the waiting list for unrelated donor transplantation.
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PMID:Clinical sensitivity to anthracyclines in PH/BCR+ acute lymphoblastic leukemia. 1050 Aug 26

A 38-year-old man with refractory follicular lymphoma underwent allogeneic BMT from an HLA-identical sibling donor. He had generalized lymphadenopathy, hepatosplenomegaly and lymphoma infiltration of the marrow, all of which disappeared within 3 months following transplantation. Six months post-transplant, progressive hepatomegaly developed in the absence of splenomegaly and lymphadenopathy, and he died from hepatic failure. Autopsy disclosed diffuse large B cell lymphoma of the liver, into which the follicular lymphoma had transformed. Future issues to be investigated should include the optimal timing of allogeneic BMT for low-grade lymphomas.
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PMID:Histologic transformation of follicular lymphoma after allogeneic bone marrow transplantation. 1101 52

Post-transplant lymphoproliferative disorder (PTLD) is uncommonly of T cell origin, especially following BMT. We describe a 13-year-old boy with severe aplastic anemia (SAA) and no evidence of Fanconi's anemia who underwent BMT at 11 years of age using CY 10 mg/kg once daily i.v. on days -5, -4, antilymphocyte globulin (ALG) 30 mg/kg once daily i.v. on days -5 approximately -3 and CsA from day -1 as conditioning. The BMT failed and he received a further peripheral blood stem cell transplant (PBSCT) 240 days after BMT. Conditioning was with CY 50 mg/kg once daily i.v. on days -5 approximately -2, and ALG 15 mg/kg once daily i.v. on days -4 approximately -2. GVHD prophylaxis included CsA and MTX. Engraftment was later confirmed by cytogenetic studies. Desquamation and ulcers of the oral mucosa and mouth angle developed in the 13th month post PBSCT. A buccal mucosa biopsy on day +524 revealed only plasmacytosis. Immunosuppressants were discontinued at that point. Generalized lymphadenopathy, prolonged fever (waxing and waning) and facial swelling developed in the 18th month post PBSCT. A neck lymph node biopsy on day +601 showed T cell lymphoma of diffuse large cell type with monoclonal TCR gamma-chain gene rearrangement. A FISH study showed that the malignant T cells were of recipient origin. EBV in situ hybridization was negative. He did not receive further treatment apart from discontinuation of immunosuppressants. He was followed up in our out-patient clinic and showed good performance 1170 days post PBSCT. We speculate that a different mechanism was operating in the pathogenesis of T cell lymphoma in this case. Risk factors include SAA and two transplants, conditioned with CY and ALG, long term use of CsA and treatment with azathioprine.
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PMID:T cell lymphoproliferative disorder following bone marrow transplantation for severe aplastic anemia. 1108 91

We report a case of Epstein-Barr virus-associated lymphoproliferative disorder (EBV-LPD) after allogeneic bone marrow transplantation (allo-BMT) from a partially mismatched related donor for acute lymphocytic leukemia, which was treated successfully by donor lymphocyte infusion (DLI). A 54-year-old woman in first complete remission from acute lymphocytic leukemia received an unmanipulated bone marrow transplant from an HLA-A 1-locus-mismatched sibling donor after preconditioning with cyclophosphamide and total body irradiation. Prophylaxis against graft-versus-host disease (GVHD) was done with tacrolimus and short-term methotrexate. Skin GVHD (grade I) occurred on day 36, but this subsided spontaneously without treatment. On day 61, rapidly progressive cervical lymphadenopathy with fever developed. Lymph node biopsy revealed lymphoid cell proliferation, which was positive for LCA, L26 and LMP-1. A diagnosis of EBV-LPD was made. After withdrawal of the tacrolimus, DLI (1 x 10(6) CD3 cells/kg) resulted in remission. This case suggests that even in the absence of risk factors such as severe GVHD, intensive immunosuppressive therapy and ATG administration, allo-BMT from an HLA 1-locus-mismatched related donor can be complicated by EBV-LPD, and that reduction of immunosuppressive therapy and DLI can be an effective treatment for it.
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PMID:[Successful donor lymphocyte infusion for Epstein-Barr virus-associated lymphoproliferative disorder after allogeneic bone marrow transplantation from an HLA 1-locus-mismatched sibling donor in a patient with acute lymphocytic leukemia]. 1180 79

A 31-year-old woman had been suffering from fever and a sore throat since January 1999, and had a left neck lymphadenopathy in December 1999. Pathological findings of the biopsied lymphnode suggested malignant lymphoma. She was finally diagnosed as having a chronic active Epstein-Barr virus(EBV) infection because of abnormal antibody titers against EBV antigens and an increased EBV load in her peripheral blood. After receiving chemotherapy consisting of CHOP and high dose cytarabine, the amount of the EBV genome decreased below the detection limit before BMT. Therefore, instead of a conventional myeloablative transplant, we performed BMT using reduced-intensity conditioning regimens consisting of fludarabine and melphalan from an HLA-identical sibling donor. After 14 months, the patient remained in complete remission. Menstruation occurred on day 83 following BMT, and the serum level of LH and FSH on day 316 were within normal range. Under these circumstances, RIST seems to be one of the curative treatments for the patients with CAEBV with minimal late side effects.
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PMID:[Chronic active Epstein-Barr virus infection treated with reduced intensity stem cell transplantation]. 1519 49

Lymphoproliferative disorders (LPD) occur often in EBV-infected patients, especially in solid-organ and haematopoietic stem cell transplant recipients. The risk of developing LPD ranges from 1 to 25% and depends on the type of transplantation. We are presenting the case of a 9-year-old boy with acute myelogenous leukaemia in second remission, who developed LPD after matched unrelated donor bone marrow transplantation (MUD BMT) not identical in two loci. On day 50 after BMT the patient presented with fever and symptoms of paronychia. Two weeks later, bilateral cervical tenderness and adenopathy and hepatosplenomegaly developed. Bone marrow biopsy confirmed continuing remission. An extensive infection workup, including bacterial, mycotic and CMV infection yielded negative results. Basing on clinical picture and suspecting LPD, EBV-PCR was performed. The patient was found to have extremely high EBV DNA levels (4.905.152 genomes/mcg) in the peripheral blood. On days 64 and 73 after BMT, the patient received two doses of rituximab (MabThera) (375 mg/m(2)) After the first dose of rituximab EBV DNA copy numbers decreased to 707.723/mcg. However the patient's general condition was worsening; 71 days after BMT increasing aplasia and symptoms of venoocclusive disease (VOD) developed. The patient received two doses of defibrotide (Novarid). Despite of intensive therapy, progressive hepatic failure and increasing pulmonary oedema led to the patient's death, on day 96 after BMT.
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PMID:[Lymphoproliferative disorder as a complication after haematopoietic stem cell transplantation]. 1953 35


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