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  • AT  (1)
  • Bone neoplasms  (1)
  • Burkitt's lymphoma  (1)
  • 1
    Digitale Medien
    Digitale Medien
    Ossäre Manifestationen beim Non-Hodgkin-Lymphom im Kindes- und Jugendalter (2000)
    Springer
    Der Radiologe 40 (2000), S. 737-744 
    Details
    ISSN: 1432-2102
    Schlagwort(e): Schlüsselwörter Lymphom ; MRI ; Keywords Lymphoma ; Bone neoplasms ; Diffusion coefficient ; ADC
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Medizin
    Beschreibung / Inhaltsverzeichnis: Abstract Purpose. Skeletal manifestation of Non-Hodgkin's lymphoma is rare in pediatric patients. Objective of the study was to determine imaging features, before and after treatment, and to correlate these features with clinical outcome. Methods. A retrospective analysis of 1246 patients from two therapy studies (NHL-BMF-90 and 95) was performed. Imaging studies of 63 patients with bone involvement of lymphoma were reevaluated. Results. Incidence of initial bone involvement in Non-Hodgkin's lymphoma was 6.8%. Distribution was best assessed by bone scan, MRI revealed larger areas of marrow involvement and detected additional lesions. Sites of prediliction were long bones of the lower extremities with epiphyseal involvement in 39%. Residual signal alterations in MRI after succussful therapy remained in 71%. Osteonecrosis after therapy was a common finding. Clinical outcome war not correlated to the presence of bone involvement. Conclusions. Since clinical outcome is not effected by bone involvement in childhood NHL, value of screening may be limited. Knowledge of imaging characteristics is mandatory for inital evaluation of primary osseous lymphomas and symptomatic lesions as well as for therapy controlls.
    Notizen: Zusammenfassung Fragestellung. Skelettale Manifestationen des Non-Hodgkin-Lymphoms sind selten. Ziel der Studie waren die Analyse charakteristischer Veränderungen in der Bildgebung vor und nach Therapie sowie die Korrelation mit dem Therapieerfolg. Methode. Die retrospektive Analyse von 2 Therapiestudien (NHL-BMF-90 und 95) schloss 1246 Patienten ein. Die bildgebenden Untersuchungen von 63 Patienten mit skelettalem Lymphombefall wurden reevaluiert. Ergebnisse. Die Inzidenz des initialen Skelettbefalls beim Non-Hodgkin-Lymphom lag bei 6,8%. Die Verteilung der Skelettherde wurde durch die Szintigraphie am sichersten erfasst, die MRT detektierte im Vergleich größere Markraumbeteiligungen sowie zusätzliche Herdbildungen im Skelett. Prädilektionsorte waren die langen Röhrenknochen der unteren Extremitäten mit epiphysärer Beteiligung in 39% der Fälle. Residuale Signalveränderungen im MRT verblieben trotz kompletter Remission in 71% der Fälle. Osteonekrosen nach Chemotherapie waren häufig. Die therapeutische Ergebnisse wurden durch das Vorhandensein eines Skelettbefalls nicht beeinflusst. Schlussfolgerungen. Aufgrund der Beobachtung, dass die Therapieergebnisse vom Nachweis eines Skelettbefalls nicht wesentlich beeinflusst werden, erscheint der Wert der Screeninguntersuchung begrenzt. Die Kenntnis des Erscheinungsbilds vom NHL in der Bildgebung ist aber für die Beurteilung der primär ossären Lymphome und symptomatischer Herdbildungen sowie in der Therapiekontrolle notwendig.
    Materialart: Digitale Medien
    URL: http://dx.doi.org/10.1007/s001170050804
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  • 2
    Digitale Medien
    Digitale Medien
    Non-Hodgkin's lymphoma in pediatric patients with chromosomal breakage syndromes (AT and NBS): Experience from the BFM trials (2000)
    Springer
    Annals of oncology 11 (2000), S. 141-145 
    Details
    ISSN: 1569-8041
    Schlagwort(e): AT ; ataxia teleangiectasia ; Nijmegen-Breakage Syndrome (NBS) ; non-Hodgkin's lymphoma (NHL)
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Medizin
    Notizen: Abstract Background:Lymphoma and leukemia are the commonest malignantdiseases in patients with chromosomal breakage syndromes and immunodeficiency(Ataxia teleangiectasia (AT) and Nijmegen breakage syndrome (NBS)). Withimproved management of infections, malignant disease is more frequentlydiagnosed and has become one of the commonest causes of death in pediatric ATand NBS. Patients and methods:In three consecutive multicenter therapytrials for pediatric non-Hodgkin's lymphoma (NHL) (NHL-BFM), 1569 patientswith newly diagnosed NHL have been registered between 1986 and 1997. Ninepatients with AT (n = 5) and NBS (n = 4) were identified andanalysed. Results:Median age of patients with AT and NBS at diagnosis ofNHL was nine years. NHL-entities differed from non-AT/NBS-patients: diffuselarge B-cell lymphomas, n = 7 (78%); ALCL, n = 1;lymphoblastic T-cell lymphoma, n = 1. Cervical nodes, paranasalsinuses and epipharynx were the sites most frequently involved. Stages were:I and II in three patients, III in five and IV in one patient. All patientsreceived polychemotherapy according to tumor-entity and stage, none receivedradiation. Dose reductions according to individual tolerance concerned mainlyethotrexate, alkylating agents and epipodophyllotoxines. One patient died oftoxic complications, two patients relapsed and died, one patient suffered fromsecond malignancy. Five of nine patients are in 1. CCR after a medianfolluow-up of five years. Conclusions:Patients with AT and NBS suffer from rare entitiesof pediatric NHL. Curative treatment is possible and should be attempted.Intensity of therapy should be adjusted to individual risk factors andtolerance. Alkylating agents, epipodophyllotoxines should be omitted, dose ofMTX should be limited to 1 g/m2. Further cooperative trials usingstandardized approaches are required.
    Materialart: Digitale Medien
    URL: http://dx.doi.org/10.1023/A:1008391923792
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  • 3
    Digitale Medien
    Digitale Medien
    Application of long PCR to detect t(8;14)(q24;q32) translocations in childhood Burkitt's lymphoma and B-ALL (1997)
    Springer
    Annals of oncology 8 (1997), S. 31-35 
    Details
    ISSN: 1569-8041
    Schlagwort(e): Burkitt's lymphoma ; c-myc ; PCR ; translocations
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Medizin
    Notizen: Abstract Background: Burkitt's lymphoma (BL) and B-ALL are characterized bychromosomal translocations juxtaposing the c-myc gene on chromosome 8to one of the immunoglobulin loci. Translocations involving the immunoglobulinheavy chain (IgH) on chromosome 14 are found in approximately75%–90% of these tumors. The breakpoint regions arelocated over a wide range on both chromosomes. Patients and methods: To detect the translocations, we developed aPCR method to generate long products. After extraction of genomic DNA (QiaAmpSystem,Qiagen, Hilden, Germany), DNA was amplified using a mixture of Taq andPwo polymerases (Boehringer Mannheim, Germany). Several primer pairs from theSµ, JH, CH1 and the Cα regions on IgH and from exon 1 and intron 1of the c-myc gene were tested in each patient. Results: Lymphoma cells from 20 children with Burkitt's lymphoma andB-ALL characterized by FAB-L3 morphology were examined. In 11/20 patients,recombinations between chromosomes 8 and 14 could be detected with our primerpairs. PCR products from 800 to 3700 bp in length were obtained reproducibly.After amplification, the products were characterized by restriction enzymedigestion, hybridization, and in part by direct sequencing. Conclusions: This PCR-based method will allow us (1) to determinethe localization of chromosomal breakpoints in primary tumor material, (2) toinvestigate whether distinct breakpoints are associated with treatmentoutcome, and (3) to detect the presence of minimal residual tumor cells duringor after therapy.
    Materialart: Digitale Medien
    URL: http://dx.doi.org/10.1023/A:1008241514611
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