Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0027819 (neuroblastoma)
 27,800 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Op18 is a highly conserved major cytosolic phosphoprotein that is expressed at high levels in acute leukemia and in neuroblastoma. In this study we present evidence pointing to a role for Op18 in cellular proliferation. Blocking of Op18 mRNA translation using antisense oligonucleotides delayed entrance of mitotically stimulated normal peripheral blood lymphocytes into the S phase. Moreover treatment of HL-60 promyelocytic leukemia cells with DMSO or PMA which induced terminal differentiation resulted in a decrease in the level of Op18 RNA and protein. Inhibition of lymphoid proliferation with cyclosporin also resulted in reduced Op18 levels.
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PMID:Involvement of OP18 in cell proliferation. 193 Feb 3

Since May 1979, 47 patients with pediatric malignancy aged 1 to 18 years (median: 7) were treated with cryopreserved autologous bone marrow transplantation (ABMT) in the department of pediatrics, National Cancer Center Hospital. The malignancies were acute non-lymphocytic leukemia (n = 8), acute lymphocytic leukemia (n = 5), osteosarcoma (n = 7), neuroblastoma (n = 6), brain tumor (n = 5), rhabdomyosarcoma (n = 4), retinoblastoma (n = 3), Ewing's sarcoma (n = 3), non-Hodgkin's lymphoma (n = 2), malignant histiocytosis (n = 1), hepatoblastoma (n = 1), malignant melanoma (n = 1) and malignant neuroepithelioma (n = 1). Conditioning regimens for solid tumors were multi-agent high-dose chemotherapy, mainly consisted of cyclophosphamide (CY) 120 mg/kg or melphalan 180mg/m2 and that for hematological malignancies were CY with fractionated total body irradiation (12 Gy). In vitro purging by 4-hydroperoxycyclophosphamide was performed in 12 leukemia patients and 5 solid tumor patients. Of the 13 patients with acute leukemia, 1 died from relapse 1 year after the unpurged marrow transplantation and 1 relapsed in the testis. Remaining 11 patients are alive in continuous complete remission with a median follow up of 30 months (range, 2 to 65 months) after transplantation. The disease-free survival rate of them was 78%. Of the 34 patients with solid tumor, 21 patients died, their cause of death were relapse in 18 and each one of infection, graft failure and brain hemorrhage. Thirteen patients are alive without disease with a median follow up of 28 months (range, 2 to 107 months) posttransplant. The longest survivor is a brain tumor girl, and there are 5 other long survivors; 2 of them are osteosarcoma and each one of rhabdomyosarcoma, Ewing's sarcoma and malignant histiocytosis. The disease-free survival rate of total 34 solid tumor patients is 29%, but that of 17 patients who received ABMT in responsive and minimum tumor residue (MTR) period was 69%. These results suggest that autologous bone marrow transplantation is an effective and tolerable treatment for poor prognostic pediatric malignancies, especially for acute leukemia and such solid tumor as that in MTR state.
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PMID:[Autologous bone marrow transplantation in pediatric cancer]. 226 Aug 67

Peripheral pancytopenia is a syndrome which allows for an early diagnosis, and although is may cover a large number of pathological entities, it can be clearly defined into three groups of illnesses which evolve with this syndromal manifestations. The first group includes non-neoplastic illnesses which include aplastic anemia, hemophagocytic syndrome associated to infection, immunological diseases and the deficiency of folates or vitamin B12. The second group includes neoplastic diseases as acute leukemia, non-Hodgkin lymphoma, and Hodgkin's lymphoma with myelofibrosis, malignant histiocytosis and non-hematological neoplasms, like the neuroblastoma and the embryonal rhabdomyosarcoma. The third group is formed by illnesses which have some similarity with neoplasms.
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PMID:[Peripheral pancytopenia]. 228 61

Normal human bone marrow cells were mixed with neuroblastoma cells from four different human cell lines, and the cell mixtures were separated by differential agglutination with soybean agglutinin (SBA). The unagglutinated cell fraction, previously shown to be highly enriched for the hematopoietic pluripotential stem cells and capable of reconstituting lethally irradiated adult patients with acute leukemia, was further fractionated by affinity chromatography on the lectin conjugated to Sepharose 6MB beads. Two independent assays, one using radiolabeling of the tumor cells and the other based on cloning of the neuroblastoma cells on agar, showed that the agglutination step alone removes 64-76% of the radiolabeled neuroblastoma cells and 85-98% of the clonogenic cells from the tumor/bone marrow cell mixture. Passage of the unagglutinated radiolabeled cells through SBA-Sepharose columns results in further purging of 28-53% of the neuroblastoma cells. Thus a combination of the two methods affords only one-log depletion for the neuroblastoma cells, compared to a three-log depletion achieved for a T-cell leukemia line CEM tested in parallel. It seems therefore that the agglutination technique, or the use of SBA-Sepharose columns, can be used only as a preliminary step for the purging of neuroblastoma cells from involved human bone marrow preparations. Staining with fluorescein isothiocyanate-conjugated SBA of nine different neuroblastoma cell lines, including the four tested in the fractionation studies, showed that more than 98% of the cells, of all the cell lines tested, specifically bind to the lectin, whereas no specific binding can be detected on the stem cell-enriched bone marrow cell fraction. However, the total number of receptors on the neuroblastoma cells is small compared to that of line CEM or normal granulocytes, which are strongly agglutinated by SBA. It seems therefore that the quantitative difference in the total number of SBA receptors is a crucial factor for purging by the agglutination technique or by affinity chromatography. Although these results show limitations to the use of both methods, this study establishes that all neuroblastoma cell lines tested express receptors for the lectin. Improved purging of neuroblastoma cells may possibly be achieved by targeting SBA-bound toxins or magnetic spheres to these receptors.
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PMID:Differential binding of soybean agglutinin to human neuroblastoma cell lines: potential application to autologous bone marrow transplantation. 241 94

The levels of circulating hematopoietic progenitor cells were measured sequentially in eight children receiving chemotherapy for acute leukemia or neuroblastoma. Significant increases in the progenitor levels (up to 50-fold in CFU-GM numbers) were observed during post-chemotherapy cytopenia in all cases, but differences among individuals in the kinetics of recovery of less committed progenitors (CFU-mix) contrasted with the synchronized-mode of expansion of committed progenitors (CFU-GM). Peripheral blood cells were collected by repeated continuous-flow leukaphereses from three of the children during post-chemotherapy expansion of the progenitor pool and were cryopreserved after fractionation procedures. Infusion of these stored cells into the patients after marrow-ablative chemotherapy established trilineage hematopoiesis. This use of stem cell rescue should be useful as an alternative to bone marrow transplantation and extends the application of cure-oriented salvage therapy to childhood cancers.
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PMID:Peripheral blood stem cell autotransplantation in treatment of childhood cancer. 256 91

Bone marrow autotransplantation involves the administration of very high doses of chemotherapy or radiation therapy, or both, followed by infusion of autologous hematopoietic stem cells. This treatment was used in the past as a salvage therapy for patients with end-stage cancers. Occasional cures in patients with chemotherapy-responsive malignancies encouraged oncologists to utilize this treatment earlier when a better result might be achieved. This has led to a substantial number of long-term disease-free survivors in non-Hodgkin's lymphoma, Hodgkin's disease, acute leukemia, and neuroblastoma. Studies are currently ongoing in the treatment of breast cancer, multiple myeloma, testicular cancer, and ovarian cancer. Important areas for future investigation include the identification of optimal criteria for patient selection and timing of the therapy, the need for infusion of hematopoietic stem cells as cloned hematopoietic growth factors become available, the identification of the most effective high-dose regimens, and the need for "purging" tumor cells from the marrow before re-infusion. Successfully addressing these issues will increasingly require large comparative trials.
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PMID:Bone marrow autotransplantation. 264 72

A patient undergoing treatment with cytotoxic chemotherapy for Hodgkin's disease developed graft versus host disease (GVHD) following a transfusion of packed red cells. This is the 28th reported patient with a malignancy who did not have a bone marrow transplant and developed GVHD after transfusion of normal blood or blood products. All patients had received cytotoxic chemotherapy prior to acquiring GVHD. The underlying malignancies included lymphoma, acute leukemia, neuroblastoma, rhabdomyosarcoma, and glioblastoma. Twenty-three of the 28 patients died of GVHD. The incidence of transfusion-related GVHD in this patient population is low but the illness is often fatal as treatment is largely ineffective. Transfusion-related GVHD can be prevented by irradiating all blood products with 1500 rad prior to administration.
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PMID:Graft versus host disease following transfusion of normal blood products to patients with malignancies. 331 50

The incidence of acute leukemia in pregnancy is low and even referral centers have limited experience. Although the short-term risks for children exposed in utero to cytotoxic agents are predictable, there is no information on long-term complications. We report here our experience in the treatment of seven cases of acute leukemia diagnosed during pregnancy, and a literature review of 51 cases published since 1975. Fifty-three patients received chemotherapy during their pregnancies. Forty-nine of the 58 cases resulted in the birth of 50 live infants. Twenty-eight infants were born prematurely, and four had low birth-weights for their gestational age. Thirty-three percent of the newborns exposed to chemotherapy in the last month of pregnancy were cytopenic at birth, but other perinatal complications were not increased. Only one child (present series) had obvious congenital malformations, and this same infant later developed a neuroblastoma arising in the adrenal gland and a papillary carcinoma of the thyroid. Follow-up data are not provided in most previously reported cases, but long-term follow-up of our cases from 1 to 17 years has shown normal growth and development and no further malignancies. A central registry is strongly advised in order to document the long-term complications arising in children exposed to chemotherapy in utero.
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PMID:Acute leukemia during pregnancy: the Toronto Leukemia Study Group experience with long-term follow-up of children exposed in utero to chemotherapeutic agents. 347 57

Autologous bone marrow transplantation (ABMT) is a new technique which is currently being evaluated in the treatment of leukemias, lymphomas, and a few solid tumors. In patients with acute leukemia (AL), high dose therapy + ABMT is of little benefit if done at time of relapse. On the other hand, when used for consolidation of remission, either with cleansed or non cleansed marrow, it may improve disease-free survival. In patients with acute myelocytic leukemia (AML) autografted during their 1st remission, the probability of remaining in remission at 2 years is 70 p. 100. It is slightly lower for patients with acute lymphocytic leukemia (ALL): 55 p. 100. The different techniques of cleansing the marrow, monoclonal antibodies, immunotoxins, drugs, are reviewed in this paper. A comparison of these techniques in term of tumor log cell kill is provided. ABMT is the best second line therapy for non-Hodgkin lymphomas (NHL), either after relapse after conventional chemotherapy or partial failure (partial remission). In these patients, the probability of remaining in remission at 3 years is about 50 p. 100. ABMT is currently under trial in the treatment of solid tumors and some success has been obtained in carcinoma of the ovary, non-seminomatous tumor of the testis, neuroblastoma, and some selected breast cancers.
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PMID:[Autograft of bone marrow for the treatment of acute leukemia: in vitro efficacy of anti-leukemic purification]. 352 33

The case histories of 72 subsequently treated patients - 44 with acute leukemia, 10 with chronic myeloid leukemia, 16 with severe aplastic anemia and 2 with neuroblastoma - were analyzed after bone marrow transplantation (BMT) with respect to pulmonary diseases. Thirty-eight patients suffered from a total of 51 pulmonary complications, which led to death in 20. Of 13 patients, 3 died of bacterial pneumonia, all of them during granulocytopenia; 2 of 6 patients died of fungal pneumonia and 2 out of 3 of a mixed bacterial-mycotic infection. Adult respiratory distress syndrome (ARDS) led to death in 2 patients. A granulocyte count under 500/microliter correlated significantly (P less than 0.002) with the fatal outcome of bacterial, fungal and ARDS pneumonia as well as with bronchitis. Viral pneumonia led to death in 8 of 9 patients; in each there was a significant correlation (P less than 0.05) with graft-versus-host disease (GvHD). Patients with repeated episodes of pulmonary illness had significantly more chronic GvHD (P less than 0.05); several of these patients displayed a reduction in helper T cells and an increase in suppressor T cells in the peripheral blood. The natural killer (NK) cells were reduced and the percentage of activated NK cell level lay between 6% and 69%. B-cells were absent or deficient. These findings explain in part the absence of specific antibody reactivity. Five of these patients also contracted GvHD-associated obstructive bronchiolitis, which did not respond to therapy. Pulmonary infiltrates of unknown origin (including idiopathic interstitial pneumonia) occurred in 8 of the patients (11.1%), with a fatal outcome in 3 patients. Significant changes (P less than 0.05) in lung function after BMT appeared in the form of reduced vital capacity (VC) increased residual volume (RV) and an increase in RV expressed as the percentage of total lung capacity. Pulmonary diseases were the most common complication and cause of death in our patients after BMT.
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PMID:Lung diseases after bone marrow transplantation. Results of a clinical, radiological, histological, immunological and lung function study. 352 53


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