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INTRODUCTION: Acute promyelocytic leukemia currently presents an excellent chance of cure with protocols based on all-trans-retinoic acid (ATRA) and anthracycline or only differentiation agents. However, high early mortality rates continue to be reported METHODS: Between 2000 and 2018, patients were enrolled and retrospectively analyzed by medical records. A modified AIDA protocol, with a 1-year shortening of the treatment duration, reduction in the number of drugs and a strategy to reduce early mortality by the postponement of the initiation of anthracyclines were employed. Overall and event-free survival rates and toxicity were analyzed RESULTS: Thirty-two patients were enrolled, of whom 56% were female, with a median age of 12 years and 34% belonged to the high-risk group. Two patients had the hypogranular variant and three had another cytogenetic alteration, in addition to the t(15;17). The median start of the first anthracycline dose was 7 days. There were two early deaths (6%) due to central nervous system (CNS) bleeding. All patients achieved molecular remission after the consolidation phase. Two children relapsed and were rescued by arsenic trioxide and hematopoietic stem cell transplantation. The presence of disseminated intravascular coagulation (DIC) at diagnosis (pâ¯=â¯0.03) was the only factor with survival impact. The five-year event-free survival (EFS) was 84% and 5-year overall survival (OS) was 90% CONCLUSION: The survival results were comparable to those found in the AIDA protocol, with a low rate of early mortality in relation to the Brazilian reality.
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Background: The safety of the new vaccines against SARS-CoV-2 have already been shown, although data on patients with polyneuropathy are still lacking. The aim of this study is to evaluate the adherence to SARS-CoV-2 vaccination, as well as the reactogenicity to those vaccines in patients affected by neuropathy. Methods: A multicentric and web-based cross-sectional survey was conducted among patients affected by neuropathy from part of South Italy. Results: Out of 285 responders, n = 268 were included in the final analysis and n = 258 of them (96.3%) were fully vaccinated. Adherence to vaccination was higher in patients with hereditary neuropathies compared to others, while it was lower in patients with anti-MAG neuropathy (all p < 0.05). The overall prevalence of adverse events (AEs) was 61.2% and its occurrence was not associated with neuropathy type. Being female and of younger age were factors associated with higher risk of AEs, while having an inflammatory neuropathy and steroids assumption were associated with a lower risk (all p < 0.05). Younger age, having had an AE, and COVID-19 before vaccination were factors associated with symptoms worsening after vaccination (all p < 0.05). (4) Conclusions: Patients with neuropathy showed a high level of adherence to COVID-19 vaccination. Safety of vaccines in patients with neuropathies was comparable to the general population and it was more favorable in those with inflammatory neuropathy.
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ABSTRACT Introduction: Acute promyelocytic leukemia currently presents an excellent chance of cure with protocols based on all-trans-retinoic acid (ATRA) and anthracycline or only differentiation agents. However, high early mortality rates continue to be reported Methods: Between 2000 and 2018, patients were enrolled and retrospectively analyzed by medical records. A modified AIDA protocol, with a 1-year shortening of the treatment duration, reduction in the number of drugs and a strategy to reduce early mortality by the postponement of the initiation of anthracyclines were employed. Overall and event-free survival rates and toxicity were analyzed Results: Thirty-two patients were enrolled, of whom 56% were female, with a median age of 12 years and 34% belonged to the high-risk group. Two patients had the hypogranular variant and three had another cytogenetic alteration, in addition to the t(15;17). The median start of the first anthracycline dose was 7 days. There were two early deaths (6%) due to central nervous system (CNS) bleeding. All patients achieved molecular remission after the consolidation phase. Two children relapsed and were rescued by arsenic trioxide and hematopoietic stem cell transplantation. The presence of disseminated intravascular coagulation (DIC) at diagnosis (p = 0.03) was the only factor with survival impact. The five-year event-free survival (EFS) was 84% and 5-year overall survival (OS) was 90% Conclusion: The survival results were comparable to those found in the AIDA protocol, with a low rate of early mortality in relation to the Brazilian reality.
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Objetivo: Avaliar o comportamento clínico e auditivo dos pacientes com doença auto-imune (DAI), em particular Artrite Reumatóide (AR) e Lúpus Eritematoso Sistêmico (LES) e comparar os achados audiométricos e às emissöes otoacústicas (EOAs) - produtos de distorçäo nestes pacientes. Casuística e Método: Foram selecionados 40 pacientes com DAI (AR e LES), sendo 27 portadores de AR e 13 de LES, 95 por cento do sexo feminino, mediana 56 anos no grupo de AR e 29 anos no grupo de LES. A avaliaçäo auditiva foi realizada através de anamnese dirigida, exame otorrinolaringológico, audiometria tonal e emissöes otoacústicas evocadas (produtos de distorçäo). Resultados: Foram estudadas 80 orelhas sendo observada alteraçäo à audiometria tonal em 19 (19/54 - 35 por cento) no grupo de AR e, em apenas quatro (4/26 - 15,4 por cento) no grupo de LES. Em relaçäo às Otoemissöes, foi detectada alteraçäo em 37 orelhas (37/54 - 68,5 por cento) do grupo de AR e em oito (8/26 - 30,7 por cento) no grupo de LES. Das 80 orelhas com DAI, 23 (28,8 por cento) apresentaram audiometria alterada e 45 (56,3 por cento) alteraçöes às otoemissöes. Conclusäo: Os autores sugerem que as emissöes otoacústicas (EOAs) produtos de distorçäo sejam mais sensíveis que a audiometria tonal na detecçäo de alteraçöes cocleares nas doenças auto-imune, fazendo-se necessário um seguimento a longo prazo para que se confirme o valor preditivo das EOAs no prognóstico audiológico dos pacientes com DAI