RESUMO

BACKGROUND AND AIMS: In high-income countries, treatment protocols for acute lymphoblastic leukemia (ALL) in children lead to a 5-year overall survival (OS) approaching 90%. There is scarce information on protocols and results of therapy from low-middle income countries (LMIC). We documented the results of treating children with ALL with two protocols in consecutive 5-year periods at a reference center in northeast Mexico. PATIENTS AND METHODS: Children ≤16 years of age diagnosed with ALL treated with two protocols were studied. Each protocol was used for 5 years; 246 children, 112 in protocol 1 and 134 in protocol 2, were included. Protocols were BFM-inspired and adapted from several regimens; protocol 2 was intended to decrease toxicity and need for hospitalization. Event-free survival (EFS) and overall survival (OS) were determined using the Kaplan-Meier method. RESULTS: In protocol 1, 103 patients (91.96%) achieved complete remission compared to 106 (79.10%) in protocol 2 (p = 0.001). The 5-year OS was 67.1% for protocol 1 vs. 55.5% for protocol 2, whereas EFS was 58.2% vs. 36.9%, respectively. Relapse occurred in 45 patients (40.17%) in protocol 1 vs. 42 (31.34%) in protocol 2 (p = 0.181). OS 1 year after relapse was 52.4% vs. 57.1%, respectively. No difference in relapse rate was documented. CONCLUSIONS: No improvement in survival rates of children with ALL from a low-income group living in a LMIC was achieved over a decade. Implementation of contemporary protocols with a high success rate is mandatory.

Assuntos

Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Leucemia-Linfoma Linfoblástico de Células Precursoras/tratamento farmacológico , Adolescente , Criança , Pré-Escolar , Intervalo Livre de Doença , Feminino , Humanos , Renda , Lactente , Masculino , México , Leucemia-Linfoma Linfoblástico de Células Precursoras/mortalidade , Recidiva , Indução de Remissão , Estudos Retrospectivos , Taxa de Sobrevida , Resultado do Tratamento

RESUMO

To document the experience of one referral service with patients diagnosed with Evans syndrome, the treatment and response and to briefly review current treatment strategies and results.METHODS: Patients enrolled in this study fulfilled criteria for Evans syndrome. Data were retrieved from the clinical files and electronic databases of the Department of Hematology, Hospital Universitario "Dr. José Eleuterio González". Treatment modalities and response and the use of additional therapies were evaluated. The literature was reviewed in the context of the clinical course of the studied patients.RESULTS: Six patients were diagnosed with Evans syndrome in the study period. Patient 1 was treated with steroids, relapsed twice and was again treated with steroids. Patient 2 treated initially with steroids plus intravenous immunoglobulin was subsequently lost to follow-up. A good response was achieved in Patients 3 and 4, who were treated with steroids plus rituximab; patient 4 also received danazol as a second-line therapy. However both relapsed and subsequently underwent splenectomy at ten and nine months, respectively. One patient, number 5, treated with steroids, danazol and rituximab did not relapse within four years of follow-up and Patient 6, who received steroids plus danazol did not relapse within three years of follow-up.CONCLUSION: Evans syndrome is an uncommon hematologic condition rarely diagnosed and not widely studied. Clinicians must have it in mind when evaluating a patient with a positive direct antiglobulin test, anemia and thrombocytopenia, since prognosis depends on its early recognition and opportune therapy, but even this leads to variable results...

Assuntos

Humanos , Masculino , Feminino , Criança , Adolescente , Adulto Jovem , Anemia Hemolítica Autoimune , Anticorpos Monoclonais , Síndrome Antifosfolipídica , Neutropenia , Trombocitopenia

RESUMO

OBJECTIVE: To document the experience of one referral service with patients diagnosed with Evans syndrome, the treatment and response and to briefly review current treatment strategies and results. METHODS: Patients enrolled in this study fulfilled criteria for Evans syndrome. Data were retrieved from the clinical files and electronic databases of the Department of Hematology, Hospital Universitario "Dr. José Eleuterio González". Treatment modalities and response and the use of additional therapies were evaluated. The literature was reviewed in the context of the clinical course of the studied patients. RESULTS: Six patients were diagnosed with Evans syndrome in the study period. Patient 1 was treated with steroids, relapsed twice and was again treated with steroids. Patient 2 treated initially with steroids plus intravenous immunoglobulin was subsequently lost to follow-up. A good response was achieved in Patients 3 and 4, who were treated with steroids plus rituximab; patient 4 also received danazol as a second-line therapy. However both relapsed and subsequently underwent splenectomy at ten and nine months, respectively. One patient, number 5, treated with steroids, danazol and rituximab did not relapse within four years of follow-up and Patient 6, who received steroids plus danazol did not relapse within three years of follow-up. CONCLUSION: Evans syndrome is an uncommon hematologic condition rarely diagnosed and not widely studied. Clinicians must have it in mind when evaluating a patient with a positive direct antiglobulin test, anemia and thrombocytopenia, since prognosis depends on its early recognition and opportune therapy, but even this leads to variable results.