RESUMO
Since 01.07.1993 to 30.09.2004, 675 children with ALL-SR were diagnosed and treated according to the modified ALL-BFM 90 protocol. Subject to statistical analysis (Kaplan-Meier method) were thus 197 children with ALL-SR treated with HD-MTX in a dose 5.0g/ m2. Among them, 21 patients failed to respond to therapy: 2 (1.0%) early deaths, 2 (1.0%) deaths during I complete remission, 16 (8.2%) relapses, 1 (0.5%) second neoplasm. Relapses occured: 12 (6.2%) in bone marrow, 2 (1.0%) in central nervous system, 1 (0.5%) in testicle and in 1 (0.5%) child combined relapse was observed. Probability rates for 11-year event free survival (EFS) was 0.80 (0.03). Application of high dose of methotrexate is safety and effective in prevention of relapses, especially meningeal and testicular involvement.
Assuntos
Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Metotrexato/administração & dosagem , Leucemia-Linfoma Linfoblástico de Células Precursoras/tratamento farmacológico , Adolescente , Criança , Pré-Escolar , Estudos de Coortes , Intervalo Livre de Doença , Relação Dose-Resposta a Droga , Feminino , Humanos , Lactente , Masculino , Polônia/epidemiologia , Leucemia-Linfoma Linfoblástico de Células Precursoras/epidemiologia , Leucemia-Linfoma Linfoblástico de Células Precursoras/mortalidade , Estudos Retrospectivos , Resultado do TratamentoRESUMO
Congenital leukaemia occurs in only 0.8% of all cases of leukaemia in children. Despite great progress in the treatment of childhood leukaemia, prognosis is still poor. This type of leukaemia must be distinguished from leukaemic reactions and transient myeloproliferative disorder. Transient myeloproliferative disorder is a rare condition in the neonatal period, connected with trisomy or other abnormalities of chromosome 21. It is characterized by high blastosis in peripheral blood and bone marrow and it usually resolves without specific therapy in 1 to 3 months. We present two cases: congenital leukaemia and transient myeloproliferative disorder. The first patient was a boy in whom congenital myelomonoblastic leukaemia (M4 in FAB classification) was diagnosed at age of 6 weeks. He was treated according to BFM-96 for acute myeloblasts leukaemia protocol, but there was no remission and he died of progressive congenital leukaemia after 4 months. The second patient was a female neonate with Down's syndrome and a cardiac defect (common atrioventricular canal) in whom hyperleukocytosis with blastosis in peripheral blood and bone marrow were detected at 2 days of age. Although no specific antileukaemic therapy was given her condition improved. At age of 3 months we observed normalisation of peripheral blood and at 5 months of age the bone marrow smear was normal. These cases confirm the difficulties in differentiation between congenital leukaemia and transient myeloproliferative disorder presented in literature. In spite of the same haematological symptoms the only difference may be detection of nonhematopoietic tissue infiltration (skin and central nervous system) commonly occurring in congenital leukaemia or the presence of trisomy and other abnormalities of chromosome 21 in transient myeloproliferative disorder.
Assuntos
Leucemia Linfoide/congênito , Leucemia Linfoide/diagnóstico , Leucemia Mieloide Aguda/congênito , Leucemia Mieloide Aguda/diagnóstico , Transtornos Mieloproliferativos/congênito , Transtornos Mieloproliferativos/diagnóstico , Diagnóstico Diferencial , Síndrome de Down/complicações , Síndrome de Down/diagnóstico , Evolução Fatal , Feminino , Humanos , Recém-Nascido , Leucemia Linfoide/terapia , Leucemia Mieloide Aguda/terapia , Masculino , Resultado do TratamentoRESUMO
Since 01.07.1993 to 30.09.2002, 640 children (48.2% girls and 51.8% boys) with ALL-SR were diagnosed and treated according to the modified ALL-BFM 90 protocol. In 29 children the treatment was intensified because of poor corticosteroid response. Subject to statistical analysis (Kaplan-Meier method) were thus 611 children with ALL-SR. Among them, 89 patients failed to respond to therapy: 10 (1.6%) early deaths, 15 (2.5%) deaths during I complete remission, 64 (10.5%) relapses. Relapses occurred: 45 (7.4%) in bone marrow, 11 (1.8%) in central nervous system, 4 (0.7%) in testicular and in 4 (0.7%) children combined relapses were observed. Probability rates for 9-year event free survival (EFS) and relapse free survival (RFS) for all patients were 0.77 (0.02) and 0.79 (0.02), respectively. Application of high dose of methotrexate is effective in prevention of relapses, especially meningeal and testicular involvement.
