RESUMO
PURPOSE: To assess the value of initial peripheral blast count in patients with acute lymphoblastic leukemia (ALL) and prednisone good response (PGR). PATIENTS AND METHODS: From January 1990 to December 1995, 403 consecutive patients with newly diagnosed ALL were enrolled in the authors' protocol 1-ALL90-BFM/HPG. Prednisone good response was defined as a blast count of less than 1,000/microL and a prednisone poor response (PPR) as a blast count of at least 1,000/microL, both in peripheral smears, after 7 days of oral prednisone (60 mg/m2 per day) and one intrathecal dose of methotrexate. In the PGR group, patients were divided into two subgroups: patients who had less than 1,000 blasts/microL at diagnosis and those with at least 1,000 blasts/microL at diagnosis. RESULTS: Three-hundred thirty-seven patients (90%) had PGR and 37 had (10%) PPR. At 5-year follow-up, event-free survival estimates were 67 +/- 3.8% and 38 +/- 8% for PGR and PPR, respectively (P = 0.0001). In the PGR group, 114 patients (34%) had an initial blast count of less than 1,000/microL and 223 (66%) had an initial blast count of at least 1,000/microL. The authors compared the clinical and laboratory characteristics of these subgroups at diagnosis and outcome and detected significant differences in white cell count, incidence of T immunophenotype, and presence of mediastinal or spleen enlargement. However, there were no differences in response to induction treatment, death in complete remission, relapses, or event-free survival probability. CONCLUSIONS: In the PGR group, regardless of the initial blast count, both subgroups had the same outcome. The PGR group with an initial blast count of at least 1,000/microL had significantly higher white cell counts. T markers, and mediastinal or spleen enlargement at diagnosis. Response to prednisone is a practical, inexpensive, and good prognostic factor in childhood ALL.
Assuntos
Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Crise Blástica/sangue , Leucemia-Linfoma Linfoblástico de Células Precursoras/tratamento farmacológico , Adolescente , Criança , Pré-Escolar , Intervalo Livre de Doença , Resistencia a Medicamentos Antineoplásicos , Feminino , Humanos , Lactente , Contagem de Leucócitos , Masculino , Metotrexato/administração & dosagem , Leucemia-Linfoma Linfoblástico de Células Precursoras/diagnóstico , Leucemia-Linfoma Linfoblástico de Células Precursoras/mortalidade , Prednisona/administração & dosagem , Prognóstico , Indução de Remissão , Taxa de Sobrevida , Resultado do TratamentoRESUMO
We report a 7-year old white girl who was admitted because of acute severe hepatic failure. Her complete blood count revealed pancytopenia and a bone marrow aspiration was consistent with acute lymphoblastic leukemia (ALL). Blasts cells were positive for cytoplasmic CD3 and cell surface T-associated markers. Viral, metabolic, immune and toxic causes for hepatic failure were ruled out. Treatment pre-phase with prednisone was started and liver function tests clearly improved after one-week therapy. However, due to her hepatic insufficiency, daily etoposide was administered orally during 15 days. On day 33 complete remission was achieved and hepatic function was normal, except for an increase in the bilirubin level which normalized on day 72. She received our current treatment for intermediate risk ALL and is still receiving continuation phase therapy, currently, with normal liver function and good tolerance to chemotherapy + 8 months after achieving complete remission.
Assuntos
Falência Hepática Aguda/diagnóstico , Falência Hepática Aguda/fisiopatologia , Leucemia-Linfoma Linfoblástico de Células Precursoras/diagnóstico , Leucemia-Linfoma Linfoblástico de Células Precursoras/fisiopatologia , Anti-Inflamatórios/administração & dosagem , Antineoplásicos Fitogênicos/administração & dosagem , Criança , Diagnóstico Diferencial , Etoposídeo/administração & dosagem , Feminino , Humanos , Falência Hepática Aguda/tratamento farmacológico , Leucemia-Linfoma Linfoblástico de Células Precursoras/tratamento farmacológico , Prednisona/administração & dosagemRESUMO
BACKGROUND: A high cure rate may be attained for locally advanced, undifferentiated nasopharyngeal carcinoma (NPC) in children, provided that a combined modality of treatment is employed. Both local and systemic therapies are necessary. Results at a single pediatric institution were analyzed. METHODS: From November 1988 to December 1997, 16 consecutive patients were treated with NPC at the Hospital Garrahan in Buenos Aires, Argentina. The authors were able to evaluate 11 patients (9 boys and 2 girls); their median age was 12 (range, 8-14) years. Chemotherapy consisted of 3 courses, every 3 weeks, of 5-fluorouracil (500 mg/m(2)) plus bleomycin (15 mg/m(2)) daily for 4 days, with cisplatin (100 mg/m(2)) added the last day. External beam radiotherapy was delivered over a median of 52 (range, 45-63) days, to a median cumulative dose to the primary site of 55 (range, 50-61.2) grays (Gy). The median dose for the lower neck area was 45 (range, 45-55.8) Gy. All patients received radiotherapy to the primary site and to the initially involved lymphoid areas, with daily single doses of 1.8 Gy (5 of 7 days per week). RESULTS: The main symptoms at onset were cervical mass (100%), epistaxis (54%), cephalalgia (36%), and trismus (36%). All cases were Stage IV (American Joint Committee on Cancer and International Union Against Cancer TNM system). Complete response was achieved in 45% of patients after initial chemotherapy. With a median follow-up of 63 (range, 23-119) months, disease free survival (with standard error [SE]) and overall survival estimates were 61% (16%) and 91% (9%), respectively, at 75 months. Acute toxicity due to therapy was tolerable. Chronic sinusitis (73%), hypothyroidism (73%), and mild (64%) or moderate (9%) neck fibrosis were detected at follow-up. CONCLUSIONS: Although this series is small, the authors concluded that NPC patients have a good chance of survival in the setting described, in spite of locally advanced disease. Chemotherapy might be useful in preventing the development of systemic metastases.
Assuntos
Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Carcinoma/terapia , Neoplasias Nasofaríngeas/terapia , Radioterapia de Alta Energia , Adolescente , Bleomicina/administração & dosagem , Carcinoma/patologia , Carcinoma/virologia , Criança , Cisplatino/administração & dosagem , Terapia Combinada , Feminino , Fluoruracila/administração & dosagem , Herpesvirus Humano 4/isolamento & purificação , Humanos , Masculino , Neoplasias Nasofaríngeas/patologia , Neoplasias Nasofaríngeas/virologia , Indução de Remissão , Análise de SobrevidaAssuntos
Aracnoide-Máter/patologia , Linfoma não Hodgkin/diagnóstico , Neoplasias Meníngeas/diagnóstico , Segunda Neoplasia Primária/diagnóstico , Pia-Máter/patologia , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Criança , Pré-Escolar , Irradiação Craniana , Feminino , Seguimentos , Humanos , Linfoma não Hodgkin/tratamento farmacológico , Linfoma não Hodgkin/radioterapia , Masculino , Neoplasias Meníngeas/tratamento farmacológico , Neoplasias Meníngeas/radioterapia , Segunda Neoplasia Primária/tratamento farmacológico , Segunda Neoplasia Primária/radioterapia , Indução de Remissão , Coluna Vertebral/efeitos da radiaçãoRESUMO
BACKGROUND: The association between t(8;21) and granulocytic sarcoma (GS) is well known, but to the authors' knowledge the prognostic significance of GS in these patients has not been defined clearly. METHODS: Between January 1990 and July 1999 174 children with acute myeloid leukemia were admitted to the study institution. Translocation (8;21) was identified in 20 patients (11.5%). Eighteen patients were evaluable for the current study and 8 presented with GS at the time of diagnosis (GS+). RESULTS: The authors defined two groups of patients: those who were GS+ and those who were GS-. One patient in the GS+ group and two patients in the GS- group died during the induction phase of the study. Complete remission was achieved in seven patients in the GS+ group and eight patients in the GS- group. Two patients developed a recurrence in the GS+ group as did one patient in the GS- group. The event free-survival probability (the standard error) was 58% (18%) in the GS+ group and 70% (14%) in the GS- group. Localization of GS was in only one site in seven patients and at multiple sites in one patient. Patients with an epidural mass received local radiotherapy (one patient) or surgery (two patients). Two of these patients developed paraplegia as sequelae: one patient after surgery and one patient after radiotherapy. One patient with orbital GS received local radiotherapy because of progressive proptosis. The remaining four patients had a complete resolution of the GS with chemotherapy only. CONCLUSIONS: In the current study of patients with t(8;21)(q22;q22), the presence of granulocytic sarcoma was not found to be an adverse prognostic factor. However, careful attention should be paid, especially to patients with an epidural site, to avoid sequelae. Chemotherapy appears to be the optimum treatment for these children.
Assuntos
Cromossomos Humanos Par 21/genética , Cromossomos Humanos Par 8/genética , Leucemia Mieloide Aguda/genética , Leucemia Mieloide/genética , Translocação Genética , Adolescente , Criança , Pré-Escolar , Progressão da Doença , Intervalo Livre de Doença , Feminino , Humanos , Leucemia Mieloide/terapia , Leucemia Mieloide Aguda/terapia , Masculino , Prognóstico , Resultado do TratamentoRESUMO
From January 1990 to August 1997, 29 consecutive patients were treated with newly diagnosed primary acute promyelocytic leukemia (APL) at the authors' Institution. Of these, 27 (16 boys and 11 girls) were evaluable. Median age at diagnosis was 6.3 (range: 1.9-15.7) years. This population was treated with two consecutive protocols: 13 patients were included in the AML-HPG-90 protocol and 14 in the AML-HPG-95. The initial treatment was the same for both protocols: an induction 8-day phase with cytarabine, idarubicin, and etoposide was followed by a consolidation with cyclophosphamide, cytarabine, 6-mercaptopurine, vincristine, doxorubicin, and prednisone. Two courses of intensification with high-dose (HD) cytarabine and etoposide were given in the first study. Only one intensification course was administered in the second study, with HD cytarabine plus idarubicin or etoposide decided by randomization. Complete remission was achieved in 67% (18/27) of cases. Mortality on induction was quite high, 30% (8/27) mainly due to hemorrhages from disseminated intravascular coagulation (DIC). The event-free survival estimate for all patients was 0.47 (SE: 0.1). From April 1994, all-trans-retinoic acid (ATRA) was administered just during the first days of the induction phase (median: 9, range: 2-27) to stop or prevent DIC. Eighteen patients received ATRA and 9 did not. Three patients developed signs of ATRA syndrome during the first days of administration but no one died due to this toxicity. The impact of a short course of ATRA on early control of DIC was studied by analyzing the number of platelet, cryoprecipitate, and fresh frozen plasma transfusions during the induction phase in both groups. No statistical differences in complete remission rate, early mortality, need of transfusion of blood components for DIC, and survival estimates could be established between patients who received ATRA and those who did not. ATRA used in a short-course schedule during induction of APL did not stop early mortality due to DIC. Moreover, survival results did not improve with this method of ATRA usage. Longer periods of ATRA administration during APL therapy are strongly recommended.
Assuntos
Leucemia Promielocítica Aguda/tratamento farmacológico , Tretinoína/administração & dosagem , Adolescente , Antineoplásicos/uso terapêutico , Antineoplásicos/toxicidade , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Protocolos de Quimioterapia Combinada Antineoplásica/toxicidade , Criança , Pré-Escolar , Citogenética , Dexametasona/uso terapêutico , Intervalo Livre de Doença , Coagulação Intravascular Disseminada/induzido quimicamente , Coagulação Intravascular Disseminada/complicações , Esquema de Medicação , Feminino , Produtos de Degradação da Fibrina e do Fibrinogênio/metabolismo , Fibrinogênio/metabolismo , Hemorragia/tratamento farmacológico , Hemorragia/etiologia , Transtornos Hemorrágicos/prevenção & controle , Humanos , Masculino , Contagem de Plaquetas , Estudos Retrospectivos , Fatores de Risco , Sobrevida , Fatores de Tempo , Tretinoína/toxicidadeRESUMO
Major histocompatibility complex (MHC) class II deficiency is a rare form of primary combined immunodeficiency that can only be corrected by stem cell transplantation. We report a 4(1/2)-year-old girl with MHC class II deficiency who underwent a related CBT due to graft failure following T cell-depleted non-identical BMT. The patient is alive and well 2 years after the second transplant. A sustained hematopoietic engraftment and a progressive immune recovery have been detected. We conclude that cord blood may be an effective source of hematopoietic stem cells for patients with immuno- deficiency disorders including diseases with a high rate of graft failure.
Assuntos
Transplante de Medula Óssea , Antígenos HLA-D/genética , Transplante de Células-Tronco Hematopoéticas , Síndromes de Imunodeficiência/terapia , Feminino , Sangue Fetal , Humanos , Síndromes de Imunodeficiência/imunologia , Lactente , Depleção Linfocítica , Linfócitos T , Transplante Homólogo , Falha de Tratamento , Resultado do TratamentoRESUMO
Entre los linfomas no-Hodgkin extranodales de cabeza y cuello se destaca un subgrupo localizado en el anillo de Waldeyer (amígdalas, adenoides, base de la lengua y tejido linfoide faríngeo posterior), con características especiales. Entre agosto de 1988 y diciembre de 1998 ingresaron al Hospital de Pediatría Juan P. Garrahan 138 pacientes evaluables con diagnóstico de linfoma no-Hodgkin B, de los cuales 8 (5,8 por ciento) estaban localizado en el anillo de Waldeyer (6 varones y 2 mujeres). En 7 pacientes se llegó al diagnóstico por biopsia del tumor (Burkitt: 6 y 1 linfoma a células grandes),en tanto que el restante presetnaba infiltración completa de médula ósea, evidenciándose en el estudio citogenético una translocación entre los cromosomas 8 y 14. Ningún paciente presentaba compromiso del sistema nervioso central. Los pacientes fueron tratados según dos protocolos sucesivos consistentes en bloques de poli-quimioterapia, con metotrexate a 2g/m2 y profilaxis del sistema nervioso central con medicación triple intratecal. Ningún paciente recibió radioterapia. Laedad media del diagnóstico fue de 7,5 (3-15) años. Todos los pacientes lograronla remisión completa, con muy buena tolerancia. La probabilidad de sobrevida libre de eventos es del 100 por ciento, con un seguimiento medio de 50 (3-112) meses. Conclusiones: 1) Se trata de una serie pequeña, con histología predominante de tipo Burkitt y estadíos localizados. 2) Tanto la óptima sobrevida, como la muy buena tolerancia, destacan la efectividad y utilidad de estos protocolos, aún con menor dosis de metotrexate que el protocolo original alemán y sin radioterapia.
Assuntos
Humanos , Masculino , Feminino , Pré-Escolar , Adolescente , Linfoma não Hodgkin/diagnóstico , Linfoma não Hodgkin/terapia , Argentina , Carcinoma/diagnóstico , Carcinoma/terapia , Linfoma de Burkitt/diagnóstico , Linfoma de Burkitt/terapia , Leucemia-Linfoma Linfoblástico de Células Precursoras/diagnóstico , Leucemia-Linfoma Linfoblástico de Células Precursoras/terapiaRESUMO
Entre los linfomas no-Hodgkin extranodales de cabeza y cuello se destaca un subgrupo localizado en el anillo de Waldeyer (amígdalas, adenoides, base de la lengua y tejido linfoide faríngeo posterior), con características especiales. Entre agosto de 1988 y diciembre de 1998 ingresaron al Hospital de Pediatría Juan P. Garrahan 138 pacientes evaluables con diagnóstico de linfoma no-Hodgkin B, de los cuales 8 (5,8 por ciento) estaban localizado en el anillo de Waldeyer (6 varones y 2 mujeres). En 7 pacientes se llegó al diagnóstico por biopsia del tumor (Burkitt: 6 y 1 linfoma a células grandes),en tanto que el restante presetnaba infiltración completa de médula ósea, evidenciándose en el estudio citogenético una translocación entre los cromosomas 8 y 14. Ningún paciente presentaba compromiso del sistema nervioso central. Los pacientes fueron tratados según dos protocolos sucesivos consistentes en bloques de poli-quimioterapia, con metotrexate a 2g/m2 y profilaxis del sistema nervioso central con medicación triple intratecal. Ningún paciente recibió radioterapia. Laedad media del diagnóstico fue de 7,5 (3-15) años. Todos los pacientes lograronla remisión completa, con muy buena tolerancia. La probabilidad de sobrevida libre de eventos es del 100 por ciento, con un seguimiento medio de 50 (3-112) meses. Conclusiones: 1) Se trata de una serie pequeña, con histología predominante de tipo Burkitt y estadíos localizados. 2) Tanto la óptima sobrevida, como la muy buena tolerancia, destacan la efectividad y utilidad de estos protocolos, aún con menor dosis de metotrexate que el protocolo original alemán y sin radioterapia. (AU)
Assuntos
Humanos , Masculino , Feminino , Pré-Escolar , Adolescente , Linfoma não Hodgkin/diagnóstico , Linfoma não Hodgkin/terapia , Argentina , Linfoma de Burkitt/diagnóstico , Linfoma de Burkitt/terapia , Leucemia-Linfoma Linfoblástico de Células Precursoras/diagnóstico , Leucemia-Linfoma Linfoblástico de Células Precursoras/terapia , Carcinoma/diagnóstico , Carcinoma/terapiaRESUMO
We describe an 8 year old boy who had received chemotherapy for an acute promyelocytic leukemia and developed a secondary leukemia 27 months after the diagnosis of this first malignancy. Blasts cells were positive for cytoplasmic markers CD22, CD3 and myeloperoxidase. Cell surface T and myeloid-associated markers were also detected. Cytogenetic study disclosed monosomy 7. The patient achieved complete remission, but relapsed 15 months later with identical immunophenotypic and cytogenetic findings. Three-lineage commitment is proved by the expression of specific criteria for myeloid, and lymphoid T and B typing. A multipotent immature progenitor must be the target of leukemogenic agents. The prognosis is obviously ominous.