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)

Six patients suffering from refractory malignancies (3 NHL, 1 MM, 1 AML, 1 neuroblastoma) received high dose of chemotherapy and autologous peripheral blood stem cell transplantation (APBSCT). The recruitment of PBSC was performed using conventional salvatage schedules of therapy. The patients received a median of 8.69 MNC/kg bw and 20.87 CFU-GM x 10(4)/kg bw. Prompt engraftment occurred in all patients and the median number of days to achieve WBC greater than 1 x 10(9)/l was 16.5 (range 7-26), PMN greater than 0.5 x 10(9)/l was 21.5 (range 6-37) and PLTs greater than 50 x 10(9)/l was 17.5 (range 4-31). Four patients achieved a complete remission. One patient (neuroblastoma) died of progressive disease after a partial response. One patient died in relapse because of drug related toxicity.
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PMID:Autologous peripheral blood stem cell transplantation in malignancies involving bone marrow. 167 14

With increasing survival rates of children grafted for different malignancies concerns about the longterm side effects of this treatment are growing. Therefore, investigations on the function of endocrine systems were conducted in a total 28 patients grafted for various reasons: ALL (N = 18), AML (N = 1), SAA (N = 3), CML(N = 4), neuroblastoma (N = 2). The results can be summarized as follows: 1. The extent of hormonal derangements is primarily dependent on the extent of irradiation prior to BMT. Integrity of hormonal systems was found in cases without irradiation (SAA) or if TBI did not exceed 3 Gy. 2. Primary hypogonadism was present in 18 patients. 3. Primary hypothyroidism was present in 2 patients. 4. Growth impairment was observed in 8 patients. In four of these cases growth hormone deficiency was the cause. In four other cases with graft-versus-host-disease and hepatic involvement SmC/IGF I levels were severely diminished. The data suggest that in most cases BMT itself has relatively few negative effects on the endocrine regulatory system. However, more detailed investigations before and after BMT will be needed to further validate these observations.
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PMID:Influence of allogeneic bone marrow transplantation on the endocrine system in children. 248 Mar 5

Pretreatment serum samples obtained at diagnosis from 89 children with various pediatric malignancies were examined for circulating immune complexes (CIC) using the [125I]Clq binding assay. The study population consisted of 35 children with acute lymphocytic leukemia (ALL), 22 children with acute non-lymphocytic leukemia (ALL), 24 with neuroblastoma (NB), and eight with osteosarcoma (OS). Concomitant quantitation of immunoglobulins was performed in 55 patients, revealing normal values for age. Increased levels of CIC at diagnosis were found in 9%, 22%, 42%, and 50% of children with ALL, AML, NB, and OS, respectively. Except for a higher proportion of CIC-positive patients observed in stage IV NB (nine of 17) compared to stage I-III NB (one of seven), no correlation was observed between initial CIC level and presenting clinical features, response to treatment, prognosis, or presence of infection. Longitudinal sampling of six NB and two OS patients did not reveal a clear relationship between disease activity and quantity of CIC. For the pediatric malignancies studied, these data demonstrate minimal value in quantitating CIC as a means of assessing disease activity or predicting response to treatment and are in contrast to the apparently adverse effect of elevated pretreatment CIC on response to therapy and survival observed in adults with ALL, AML, and OS.
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PMID:Circulating immune complexes in childhood malignancies: a Pediatric Oncology Group study. 658 17

We reported the experience of peripheral blood stem cell transplantation (PBSCT) performed in adult patients with hematological malignancies and solid tumors. After myelosuppressive chemotherapy, peripheral blood stem cells were collected using a Blood Cell Separator (CS-3000) during bone marrow recovery and subsequently cryopreserved in 17 patients (9: malignant lymphoma; 2: ALL; 2: AML; 2: multiple myeloma; 2: solid tumors). In 28 apheresis cases, the collected number of granulocyte/macrophage progenitors (CFU-GM) was more than 5 x 10(5)/kg BW in 17 apheresis cases and ranged between 2 and 5 x 10(5)/kg BW in 4 of such cases. Eleven patients (7: malignant lymphoma; 1: ALL; 1: AML; 1: multiple myeloma; 1: neuroblastoma) underwent PBSCT following myeloablative chemotherapy. The infused number of CFU-GM ranged between 0.6 and 18.1 x 10(5)/kg BW. In 7 patients, more than 5 x 10(5) CFU-GM/kg BW were infused. The median time to reach 500 neutrophils/microliter or 50,000 platelets/microliter was 10 (range: 8-17) and 20 (range: 8-63) days, respectively. One patient died from sepsis before hematologic recovery occurred. Eight patients are alive with no evidence of active disease for 7-19 months after PBSCT. When the infused number of CFU-GM is more than 2 x 10(5)/kg BW, PBSCT following myeloablative chemotherapy seems to be safe and useful treatment.
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PMID:[Peripheral blood stem cell transplantation in adult patients]. 768 Aug 48

From April 1988 to May 1993, 71 patients (32 acute myelogenous leukaemia [AML], 24 acute lymphoblastic leukaemia [ALL], 7 Hodgkin's disease [HD], 5 non-Hodgkin lymphoma [NHL], 2 neuroblastoma, 1 chronic myelogenous leukaemia [CML]) were treated with myeloablative therapy followed by reinfusion of cryopreserved autologous bone marrow (ABMT). The majority of patients with acute leukaemia were in first complete remission (CR), while 11 AML patients and 9 ALL patients were in advanced stage of the disease (> I CR or relapse). The BM was reinfused without purging. The conditioning regimen for all ALL and proportion of AML patients consisted of cyclophosphamide (CY) 120 mg/kg and fractionated total body irradiation (TB) in a total dose of 12 Gy. 18 AML patients received busulfan 16 mg/kg instead of TBI. Leukaemia-free survival (LFS) for first CR AML patients was 48% at 43 months with the median follow-up of 17 months. Probability of relapse was 44%. LFS for advanced AML was only 9% and the probability of relapse 89%. LFS for first CR ALL patients was 72% at 53 months with the median follow-up of 15 months, while probability of relapse was only 23%. For advanced ALL, LFS was 32% at 33 months and probability of relapse 64%. Probability of toxic death for first CR patients was 11%. We found a predictive value of viability testing and in vitro CFU-GM assay for haematologic recovery after ABMT. We conclude that ABMT with cryopreserved BM is a relatively safe method for consolidation therapy of AL. The results of treatment are encouraging.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Autologous bone marrow transplantation for haematological malignancies--experiences of the centre of Zagreb. 776 56

If residual cancer cells in harvested bone marrow could be marked and subsequently detected in patients at relapse, valuable information would be obtained about the source of recurrent disease after autologous marrow transplantation. If normal progenitor cells were also marked, the study would provide useful data on the susceptibility of these human cells to gene transfer and their capacity to express newly introduced genes. We transferred the neomycin-resistance gene (NeoR) into bone marrow cells harvested from 20 children with acute myeloid leukemia (n = 12) or neuroblastoma (n = 8) in clinical and cytological remission using a retrovirus vector. The cells were then returned to the patients as part of an autologous bone marrow transplantation protocol. Two AML and three neuroblastoma patients have relapsed. In all, the resurgent cells contained the NeoR marker by analysis with PCR. These results prove that so-called remission marrow can contribute to relapse in patients who receive autologous transplants. The gene marking technique is now being used to evaluate techniques of pretransplant purging.
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PMID:Gene marking and autologous bone marrow transplantation. 802 95

We have used gene marking to investigate the mechanism of relapse and biology of reconstitution following bone marrow transplantation (BMT). The rationale for our initial protocols was to learn if residual malignant cells in autologous marrow contribute to subsequent relapse. Marked malignant cells were found at the time of relapse in 6/8 patients relapsing after autologous BMT for AML or neuroblastoma showing the infused marrow contributed to disease recurrence. Modifications of this marker approach with two distinguishable vectors are now being used to compare the efficacy of purging techniques. We were also able to evaluate gene transfer to normal progenitors and demonstrated that the marker gene was expressed for up to 36 months. Gene marking is also being used to trace the fate of EBV-specific CTLs that we are administering to recipients of allogeneic BMT and has provided evidence of persistence of adoptively transferred CTL for up to 10 months.
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PMID:Use of gene marking in bone marrow transplantation. 870 35

Primary congenital glaucoma (gene symbol: GLC3) is an ocular disorder that occurs for 0.01-0.04% of blind people. In the majority of familial cases reported so far, this condition is inherited as an autosomal recessive trait. We have recently used a group of 17 GLC3 families with a minimum of two affected offspring and consanguinity in most of the parental generation and mapped the first GLC3 locus (GLC3A) to the 2p21 region. Six families did not show any linkage to the GLC3A locus and thus provided evidence for genetic heterogeneity of this disorder. A total of eight families unlinked to the 2p21 region were used to search for the chromosomal location of the second GLC3 locus. Herein, we describe mapping of a new locus (designated GLC3B) for primary congenital glaucoma to the short arm of chromosome 1 (1p36.2-36.1) that is situated centromeric to the neuroblastoma and Charcot-Marie-Tooth type 2A (CMT2A) loci. A total of 17 DNA markers were genotyped from this region of chromosome 1. Four families showed no recombination with the two markers D1S2834 and D1S402 with a maximum lod score of 4.510 and 4.157 respectively. Pairwise and multipoint linkage analysis and inspection of the haplotypes revealed that the remaining four families are not linked to this part of chromosome 1, thus providing further evidence that at least one more locus for the autosomal recessive form of GLC3 must exist in the genome. Based on the recombination events, the overall linkage map of this region is: tel-D1S1192-D1S1635-D1S1193 - (D1S1597/-D1S489/D1S228)- [GLC3B/D1S2834/D1S402] - (D1S1176/D1S507/D1S407) - D1S2728-(MFAP2/D1S170) - D1S1368 - D1S436-D1S1592-cen.
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PMID:A second locus (GLC3B) for primary congenital glaucoma (Buphthalmos) maps to the 1p36 region. 884 41

Manipulation of cholesterol metabolism open several possibilities of interfering with the growth of malignant cells. Deprivation of cholesterol decreases the velocity of growth and alters the composition of the cell membrane. The high requirement for LDL of malignant cells can be utilized for drug targeting. Proliferation assays were performed with neuroblastoma cells and cell lines of acute myeloid leukemia deprived of cholesterol by inhibition of HMG-CoA-reductase or culture in LDL-deficient medium. The cholesterol content of the cell membrane when reduced to 50% had no effect on the toxicity of LAK-cells but the toxicity of the fluorescent dye merocyanine MC 540 was enhanced two-fold. LDL-mediated drug targeting to AML cells was performed with oxidized LDL and showed toxic reactions. These results proved that cholesterol deprivation could be used to support some therapeutic approaches.
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PMID:Cholesterol based antineoplastic strategies. 913 62

To characterize the breakpoint in a neuroblastoma patient with a constitutional rearrangement we have constructed a yeast artificial chromosome (YAC) contig extending approximately 6 Mbp in the chromosome 1p22 region that spans the D1S435 and D1S236 loci. This contig has been confirmed by the coincidence of a number of markers in different overlapping YACs. For several of these YACs the overlap was demonstrated following the isolation and sequencing of end clones from which STS markers were generated. The majority of the YACs have been shown not to be chimeric either through the analysis of somatic cell hybrids or fluorescence in situ hybridization. Following the establishment of the contig we have been able to construct a physical map of the region that incorporates six STS and three newly assigned eSTS markers. The generation of this physical map has allowed the reordering of markers in the genetic linkage map for 1p. The physical order is; tel-D1S435-D1S188-D1S424-D1S236-D1D415- D1S420. With the reordering of D1S435 we have been able to join this contig with another reported previously, thereby generating a well characterized 15 Mbp YAC contig in the 1p22-31 region. The 6 Mbp contig described here spans the chromosome 1 constitutional translocation break-point seen in a patient with a t(1;10)(p22;q21) and who had a stage 4S neuroblastoma. YAC fragmentation has been used to define a 200 Kb region within this contig containing the 1p22 breakpoint. Restriction enzyme analysis demonstrates that there are three NotI sites in this region, one of which lies close to the translocation breakpoint site.
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PMID:Molecular characterization of the 1p22 breakpoint region spanning the constitutional translocation breakpoint in a neuroblastoma patient with a t(1;10)(p22;q21). 940 74


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