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  • AT  (1)
  • Acute lymphoblastic leukaemia  (1)
  • Allograft  (1)
  • Chemotherapy  (1)
  • 1
    ISSN: 1569-8041
    Keywords: AT ; ataxia teleangiectasia ; Nijmegen-Breakage Syndrome (NBS) ; non-Hodgkin's lymphoma (NHL)
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: 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.
    Type of Medium: Electronic Resource
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  • 2
    Electronic Resource
    Electronic Resource
    Springer
    Annals of hematology 79 (2000), S. 345-354 
    ISSN: 1432-0584
    Keywords: Key words Management ; Chronic myelogenous leukemia ; Autograft ; Allograft ; Bone marrow transplantation
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract The management of chronic myelogenous leukemia (CML) has become complex due to the availability of improved diagnostic procedures and life-prolonging or even curative treatment strategies that are more successful the earlier they are applied in the course of the disease. This is true for allogeneic bone-marrow transplantation, treatment with interferon α (IFN) and Philadelphia-negative stem-cell collections for autografting. Outcome differs according to risk profiles of patients at diagnosis. In addition, molecular techniques for the detection of the BCR-ABL fusion gene or its products, such as the reverse-transcriptase polymerase chain reaction (PCR), Southern blot analysis, or fluorescence in situ hybridization, facilitate accurate diagnosis and the monitoring of residual disease. They allow the individualization of treatment such as early infusion of donor lymphocytes if molecular relapse is detected after allografting, or discontinuation of IFN in the presence of very low BCR-ABL transcript levels). The availability of real-time PCR devices further improves and accelerates the diagnosis and monitoring of residual disease. This article addresses recent developments in drug therapy and allografting, including treatment intensification with low-dose ara C or intensive chemotherapy followed by autografting, introduction of new drugs (such as homoharringtonine or tyrosine kinase inhibitor STI571), progress with unrelated donor transplantations, use of peripheral blood stem cells for allografting, and transplantation without myeloablative conditioning. Tradeoffs between the treatment options will be discussed in the context of the evidence-based guidelines for treating CML, as recently published by the American Society of Hematology. Finally, the new competence network on acute and chronic leukemias will be introduced.
    Type of Medium: Electronic Resource
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  • 3
    Electronic Resource
    Electronic Resource
    Springer
    European journal of pediatrics 151 (1992), S. S50 
    ISSN: 1432-1076
    Keywords: Acute lymphoblastic leukaemia ; Bone marrow transplantation ; Chemotherapy ; Risk factors
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Twenty-five years ago over 90% of children with acute lymphoblastic leukaemia (ALL) died of this disease. Dramatic improvement has been achieved since then by employing risk-adapted, aggressive polychemotherapy protocols. More than 90% of children with ALL treated according to, for example BFM-protocols, have nowadays cure rates in the range of 70%–80%. However, 10% of patients do not initially respond adequately to standard induction chemotherapy. They are characterized by distinct chromosomal abnormalities such as translocation (9; 22) or combinations of early treatment failure and other risk factors as cytogenetic abnormalities, lineage-specific surface markers or tumour load at diagnosis. In this group of patients in first complete remission and certainly in the vast majority of relapsed patients, allogeneic bone marrow transplantation (BMT) has evolved as an alternative approach allowing further intensification of myeloablation and the introduction of an additional antileukaemic alloreactivity. Nevertheless, the decision for a marrow transplant in children has to be made very carefully because of a significant increase in treatment related mortality and BMT-specific risks like acute and chronic graft-versus-host disease with a critical iatrogenic chronic morbidity. This is even more evident, if mismatched or unrelated transplants are being considered. The indications for one or the other treatment modality according to the current BFM strategy are discussed.
    Type of Medium: Electronic Resource
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