Risk factors of death or chronic renal replacement therapy requirements in patients with thrombotic microangiopathies without ADAMTS-13 deficiency.

Thrombotic microangiopathy (TMA), characterized by microangiopathic hemolytic anemia, thrombocytopenia, and multisystem organ dysfunction, is a life-threatening disease. Patients with TMA who do not exhibit a severe ADAMTS-13 deficiency (defined as a disintegrin-like and metalloprotease with thrombospondin type 1 motif no. 13 activity ≥10%: TMA-13n) continue to experience elevated mortality rates. This study explores the prognostic indicators for augmented mortality risk or necessitating chronic renal replacement therapy (composite outcome: CO) in TMA-13n patients. We included 42 TMA-13n patients from January 2008 to May 2018. Median age of 41 years and 60% were female. At presentation, 62% required dialysis, and 57% warranted intensive care unit admission. CO was observed in 45% of patients, including a 9-patient mortality subset. Multivariate logistic regression revealed three independent prognostic factors for CO: early administration of eculizumab (median time from hospitalization to eculizumab initiation: 5 days, range 0-19 days; odds ratio [OR], 0.14; 95% confidence interval [CI], 0.02-0.94), presence of neuroradiological lesions (OR, 6.67; 95% CI, 1.12-39.80), and a PLASMIC score ≤4 (OR, 7.39; 95% CI, 1.18-46.11). In conclusion, TMA-13n patients exhibit a heightened risk of CO in the presence of low PLASMIC scores and neuroradiological lesions, while early eculizumab therapy was the only protective factor.

Plasma from patients undergoing allogeneic hematopoietic stem cell transplantation promotes NETOSIS in vitro and correlates with inflammatory parameters and clinical severity.

Introduction: NETosis, the mechanism by which neutrophils release extracellular traps (NETs), is closely related to inflammation. During the allogeneic hematopoietic stem cell transplantation (allo-HSCT), different stimuli can induce NETs formation. Inflammation and endothelial injury have been associated with acute graft-versus-host disease (aGVHD) and complications after allo-HSCT. We focus on the study of NETosis and its relation with cytokines, hematological and biochemical parameters and clinical outcomes before, during and after allo-HSCT. Methods: We evaluate the capacity of plasma samples from allo-HSCT patients to induce NETosis, in a cell culture model. Plasma samples from patients undergoing allo-HSCT had a stronger higher NETs induction capacity (NETsIC) than plasma from healthy donors throughout the transplantation process. An optimal cut-off value by ROC analysis was established to discriminate between patients whose plasma triggered NETosis (NETs+IC group) and those who did not (NETs-IC group). Results: Prior to conditioning treatment, the capacity of plasma samples to trigger NETosis was significantly correlated with the Endothelial Activation and Stress Index (EASIX) score. At day 5 after transplant, patients with a positive NETsIC had higher interleukin (IL)-6 and C-reactive protein (CRP) levels and also a higher Modified EASIX score (M-EASIX) than patients with a negative NETsIC. EASIX and M-EASIX scores seek to determine inflammation and endothelium damage, therefore it could indicate a heightened immune response and inflammation in the group of patients with a positive NETsIC. Cytokine levels, specifically IL-8 and IL-6, significantly increased after allo-HSCT with peak levels reached on day 10 after graft infusion. Only, IL-10 and IL-6 levels were significantly higher in patients with a positive NETsIC. In our small cohort, higher IL-6 and IL-8 levels were related to early severe complications (before day 15 after transplant). Discussion: Although early complications were not related to NETosis by itself, NETosis could predict overall non-specific but clinically significant complications during the full patient admission. In summary, NETosis can be directly induced by plasma from allo-HSCT patients and NETsIC was associated with clinical indicators of disease severity, cytokines levels and inflammatory markers.

La empatía de los enfermeros con los pacientes en los hospitales públicos / Working nurses' empathy with patients in public hospitals / A empatia dos enfermeiros com os pacientes em hospitais públicos

Objetivo: determinar los niveles de empatía en profesionales de enfermería de un hospital de alta complejidad, relacionar la edad con la empatía (y cada una de sus dimensiones), y establecer si existen diferencias entre estos niveles según el tipo de jornada laboral. Método: diseño comparativo, correlacional y transversal. La muestra utilizada (n=271) constituyó el 40,9% del total de profesionales de enfermería. Se estudiaron las propiedades psicométricas de la Escala de Empatía de Jefferson para Profesionales de la Salud. Se calcularon estadísticos descriptivos: media y desviación estándar. La asociación entre empatía y edad se estimó mediante ecuaciones de regresión y significancia estadística de los coeficientes de regresión, luego de evaluar el tipo de curva mediante análisis de varianza. Resultados: se identificó el modelo subyacente de las tres dimensiones de la empatía. Los valores de los estadísticos descriptivos observados fueron relativamente bajos en empatía y sus dimensiones. Los niveles de empatía no se asociaron con el rango de edad. No se encontraron diferencias en la empatía entre los tipos de horarios de trabajo. Se encontró variabilidad en las dimensiones: "cuidado compasivo" y "ponerse en los zapatos del paciente". Conclusión: estos resultados muestran que los niveles de empatía observados pueden implicar un desempeño deficiente en el cuidado empático de los pacientes.

Objective: to determine the levels of empathy in professional nurses of a high-complexity hospital, to relate age to empathy (and each one of its dimensions), and to establish if there are differences between these levels according to the type of working schedules. Method: comparative, correlational and cross-sectional design. The sample used (n=271) constituted 40.9% of the total number of nursing professionals. Psychometric properties of the Jefferson Scale of Empathy for Health Professionals were studied. Descriptive statistics were calculated: mean and standard deviation. The association between empathy and age was estimated using regression equations and statistical significance of the regression coefficients, after evaluating the type of curve using variance analysis. Results: the underlying model of three dimensions of empathy was identified. The values of the descriptive statistics observed were relatively low in empathy and its dimensions. Empathy levels were not associated with the age range. No differences in empathy were found between the types of work schedules. Variability was found in the dimensions: "compassionate care" and "Walking on the patient's shoes". Conclusion: these results show that the levels of empathy observed may imply a deficient performance in empathetic care for patients.

Objetivo: determinar os níveis de empatia em enfermeiros profissionais de um hospital de alta complexidade, relacionar a idade com a empatia (e cada uma das suas dimensões) e verificar se existem diferenças entre esses níveis, de acordo com o tipo de horário de trabalho. Método: delineamento comparativo, correlacional e transversal. A amostra utilizada (n=271) constituiu 40,9% do total de profissionais de enfermagem. Foram estudadas as propriedades psicométricas da Escala de Empatia de Jefferson para Profissionais da Saúde. Foram calculadas estatísticas descritivas: média e desvio padrão. A associação entre empatia e idade foi estimada por meio de equações de regressão e significância estatística dos coeficientes de regressão, após avaliação do tipo de curva por meio de análise de variância. Resultados: o modelo subjacente de três dimensões de empatia foi identificado. Os valores das estatísticas descritivas observados foram relativamente baixos em empatia e suas dimensões. Níveis de empatia não foram associados com a faixa etária. Não foram encontradas diferenças de empatia entre os tipos de horários de trabalho. Foi encontrada variabilidade nas dimensões: "cuidado compassivo" e "colocar-se no lugar do paciente". Conclusão: esses resultados mostram que os níveis de empatia observados podem implicar em um desempenho deficiente no atendimento empático aos pacientes.

Working nurses' empathy with patients in public hospitals. / La empatía de los enfermeros con los pacientes en los hospitales públicos.

OBJECTIVE: to determine the levels of empathy in professional nurses of a high-complexity hospital, to relate age to empathy (and each one of its dimensions), and to establish if there are differences between these levels according to the type of working schedules. METHOD: comparative, correlational and cross-sectional design. The sample used (n=271) constituted 40.9% of the total number of nursing professionals. Psychometric properties of the Jefferson Scale of Empathy for Health Professionals were studied. Descriptive statistics were calculated: mean and standard deviation. The association between empathy and age was estimated using regression equations and statistical significance of the regression coefficients, after evaluating the type of curve using variance analysis. RESULTS: the underlying model of three dimensions of empathy was identified. The values of the descriptive statistics observed were relatively low in empathy and its dimensions. Empathy levels were not associated with the age range. No differences in empathy were found between the types of work schedules. Variability was found in the dimensions: "compassionate care" and "Walking on the patient's shoes". CONCLUSION: these results show that the levels of empathy observed may imply a deficient performance in empathetic care for patients. (1) The levels of empathy are low in the nursing professionals studied. (2) These levels are not associated with age and type of work performed. (3) Low levels of empathy could imply a negative alteration of humanized attention.

t(10;12)(q24;q15): A new cytogenetic marker in hematological malignancies.

Cytogenetic studies have played a crucial role in the discovery of genes involved in several diseases. In the field of oncohematology, cytogenetics is still necessary for the classification and prognosis of many diseases. Here we report a new recurrent chromosome translocation, t(10;12)(q24;q15), in two patients with different hematological malignancies: myelodysplastic syndrome with excess blasts (MDS-EB), and myelofibrosis (MF) secondary to essential thrombocythemia (ET). The chromosome alteration was observed as a sole karyotype change in the patient with MDS-EB, both at the initial diagnosis and following progression to MDS-EB2. A putative HMGA2-KLLN rearrangement by RNA-sequencing was detected in this patient. The patient with ET, had a normal karyotype at diagnosis and the t(10;12)(q24;q15) translocation emerged as a sole cytogenetic alteration after transformation, and when MF was evident. We reviewed the literature to determine whether this chromosome abnormality had previously been described in other hematological patients and found two cases: an aggressive T-cell lymphoblastic lymphoma (T-LBL) and a case of transformed chronic myeloproliferative syndrome (CMS), in both of which t(10;12)(q24;q15) was also the only karyotype change. The clinical evolution of all four cases suggested that t(10;12)(q24;q15) is associated with a poor outcome in oncohematological patients.

Impact of Blood Eosinophil Variability in Asthma: A Real-Life Population Study.

Rationale: Blood eosinophil count predicts response to inhaled corticosteroids and specific biologic therapies in selected patients with asthma. Despite this important role, fundamental aspects of eosinophil behavior in asthma have not been studied. Objectives: To investigate the behavior of blood eosinophils in a population, comparing their distribution with the general population and studying their intraindividual variability over time in relation to hospital episodes (emergency department visits and hospitalizations) in clinical practice. Methods: The distribution and variability of 35,703 eosinophil determinations in 10,059 stable patients with asthma were investigated in the MAJORICA (Majorca Real-Life Investigation in COPD and Asthma) cohort. Eosinophil distribution in the asthma population was compared with a control sample from the general population of 8,557 individuals. Eosinophil variability and hospital episodes were analyzed using correlations, receiver operating characteristic (ROC) curves, and multiple regression analysis. We defined the Eosinophil Variability Index as (Eosmax - Eosmin / Eosmax) × 100%. The findings of the asthma population were retested in an external well-characterized asthma cohort. Results: The eosinophil count values and variability were higher in the asthma population than in the general population (P < 0.001). Variability data showed a better association with hospital episodes than the counting values. An Eosinophil Variability Index ⩾50% was a better predictor for any hospital episode than any of the absolute counting values. These results were validated in the external cohort. Conclusions: The eosinophil variability in patients with asthma better identifies the risk of any hospital episode than the absolute counting values currently used to target specific treatments.

Estimated prevalence of COVID-19 in Brazil with probabilistic bias correction.

Using data collected by the Brazilian National Household Sample Survey - COVID-19 (PNAD-COVID19) and semi-Bayesian modelling developed by Wu et al., we have estimated the effect of underreporting of COVID-19 cases in Brazil as of December 2020. The total number of infected individuals is about 3 to 8 times the number of cases reported, depending on the state. Confirmed cases are at 3.1% of the total population and our estimate of total cases is at almost 15% of the approximately 212 million Brazilians as of 2020. The method we adopted from Wu et al., with slight modifications in prior specifications, applies bias corrections to account for incomplete testing and imperfect test accuracy. Our estimates, which are comparable to results obtained by Wu et al. for the United States, indicate that projections from compartmental models (such as SEIR models) tend to overestimate the number of infections and that there is considerable regional heterogeneity (results are presented by state).

Outcomes of patients with chronic myelomonocytic leukaemia treated with non-curative therapies: a retrospective cohort study.

BACKGROUND: Approval of hypomethylating agents in patients with chronic myelomonocytic leukaemia is based on trials done in patients with myelodysplastic syndromes. We aimed to investigate whether hypomethylating agents provide a benefit in subgroups of patients with chronic myelomonocytic leukaemia compared with other treatments. METHODS: For this retrospective cohort study, data were retrieved between Nov 30, 2017, and Jan 5, 2019, from 38 centres in the USA and Europe. We included non-selected, consecutive patients diagnosed with chronic myelomonocytic leukaemia, who received chronic myelomonocytic leukaemia-directed therapy. Patients with acute myeloid leukaemia according to 2016 WHO criteria at initial diagnosis (ie, ≥20% blasts in the bone marrow or peripheral blood) or with unavailability of treatment data were excluded. Outcomes assessed included overall survival, time to next treatment, and time to transformation to acute myeloid leukaemia. Analyses were adjusted by age, sex, platelet count, and Chronic myelomonocytic leukaemia-Specific Prognostic Scoring System (CPSS). Patients were grouped by first received treatment with either hydroxyurea, hypomethylating agents, or intensive chemotherapy, and stratified by risk according to blast count, French-American-British subtype, CPSS, WHO 2016 subtype, and the eligibility criteria of the DACOTA trial (NCT02214407). FINDINGS: 949 patients diagnosed with chronic myelomonocytic leukaemia between April 13, 1981, and Oct 26, 2018, were included. Median follow-up was 23·4 months (IQR 11·5-42·3) from diagnosis and 16·2 months (6·6-31·6) from start of first-line treatment. 412 (43%) of 949 patients received hypomethylating agents as first treatment, 391 (41%) hydroxyurea, and 83 (9%) intensive chemotherapy. Adjusted median overall survival for patients treated with hydroxyurea versus hypomethylating agents was 15·6 months (95% CI 13·1-17·3) versus 20·7 months (17·9-23·4); hazard ratio (HR) 1·39 (1·17-1·65; p=0·0002) and 14·0 months (9·8-17·2) versus 20·7 months (17·9-23·4; HR 1·55 [1·16-2·05]; p=0·0027) for those treated with intensive chemotherapy versus hypomethylating agents. In patients with myeloproliferative chronic myelomonocytic leukaemia (myeloproliferative CMML), median overall survival was 12·6 months (10·7-15·0) versus 17·6 months (14·8-21·5; HR 1·38 [1·12-1·70]; p=0·0027) for patients treated with hydroxyurea versus hypomethylating agents, and 12·3 months (8·4-16·6) versus 17·6 months (14·8-21·5; HR 1·44 [1·02-2·03]; p=0·040) for intensive chemotherapy versus hypomethylating agents. Hypomethylating agents did not confer an overall survival advantage for patients classified as having lower-risk disease (ie, myelodysplastic chronic myelomonocytic leukaemia with <10% blasts, CMML-0, or lower-risk CPSS). INTERPRETATION: These data suggest hypomethylating agents as the preferred therapy for patients with higher-risk chronic myelomonocytic leukaemia and those with myeloproliferative CMML. Our findings also suggest that CPSS is a valuable tool to identify patients who are most likely to benefit from hypomethylating agents. Further evidence from prospective cohorts would be desirable. FUNDING: The Austrian Group for Medical Tumor Therapy.

Estimated prevalence of COVID-19 in Brazil with probabilistic bias correction / Prevalência estimada de COVID-19 no Brasil com correção probabilística de vieses / Prevalencia estimada de COVID-19 en Brasil con corrección probabilística de sesgo

Abstract: Using data collected by the Brazilian National Household Sample Survey - COVID-19 (PNAD-COVID19) and semi-Bayesian modelling developed by Wu et al., we have estimated the effect of underreporting of COVID-19 cases in Brazil as of December 2020. The total number of infected individuals is about 3 to 8 times the number of cases reported, depending on the state. Confirmed cases are at 3.1% of the total population and our estimate of total cases is at almost 15% of the approximately 212 million Brazilians as of 2020. The method we adopted from Wu et al., with slight modifications in prior specifications, applies bias corrections to account for incomplete testing and imperfect test accuracy. Our estimates, which are comparable to results obtained by Wu et al. for the United States, indicate that projections from compartmental models (such as SEIR models) tend to overestimate the number of infections and that there is considerable regional heterogeneity (results are presented by state).

Resumo: Estimamos o efeito da subnotificação de casos de COVID-19 no Brasil até dezembro de 2020, com base nos dados coletados pela Pesquisa Nacional de Amostra de Domicílios sobre COVID-19 (PNAD-COVID19) e a modelagem semi-bayesiana desenvolvida por Wu et al. O número total de indivíduos infectados é cerca de 3 a 8 vezes o número de casos notificados, a depender do estado do país. No final de 2020, os casos confirmados representavam 3,1% da população total, enquanto nossa estimativa aponta para quase 15% dos cerca de 212 milhões de brasileiros no mesmo período. O método de Wu et al., que adotamos com pequenas modificações nas especificações, aplica correções de vieses para compensar pela testagem incompleta e pela acurácia imperfeita dos testes. Nossas estimativas, que são comparáveis aos resultados obtidos por Wu et al. para os Estados Unidos, indicam que projeções a partir de modelos compartimentais (tais como modelos SEIR) tendem a superestimar o número de infecções, e que há uma heterogeneidade regional considerável (resultados apresentados por estado).

Resumen: Usando los datos recogidos por la Encuesta Nacional por Muestra de Domicilios - COVID-19 (PNAD-COVID19) y un modelado semibayesiano desarrollado por Wu et al., hemos estimado el efecto del subregistro de casos de COVID-19 en Brasil en diciembre de 2020. El número total de individuos infectados es de entre 3 a 8 veces más el número de casos informados, dependiendo del estado. Los casos confirmados son un 3,1% del total de población y nuestra estimación del total de casos es al menos un 15% de aproximadamente 212 millones de brasileños en 2020. El método que se tomó fue el de Wu et al., con leves modificaciones en las especificaciones previas, es aplicable a las correcciones de sesgo para tener en cuenta los test incompletos y la imprecisión de los tests. Nuestras estimaciones, que son comparables a los resultados obtenidos por Wu et al. para los Estados Unidos, indican las proyecciones de los modelos compartimentales (tales como los modelos SEIR), que tienden a sobreestimar el número de infecciones, así como la considerable heterogeneidad regional (los resultados se presentan por estado).

Rhabdomyosarcoma debut masquerading as acute lymphoblastic leukemia: A case report and review of the literature.

Bone marrow infiltration by alveolar rhabdomyosarcoma is uncommon, some cases can mimicry acute leukemia at presentation and mislead the diagnosis. The integration of diagnostics tests and techniques in uncommon malignancies is important to suspect and reach the diagnosis and avoid delay on treatment. We report a case of alveolar rhabdomyosarcoma bone marrow infiltration associated with hemophagocytosis and cell cannibalism, mimicking acute leukemia at presentation.

Brumadinho: entre a prudência e a probabilidade, a tragédia / Brumadinho: between prudence and probability, tragedy

Será possível, em algum momento no futuro, explicar com exatidão o que aconteceu no caso do rompimento da barragem de Brumadinho? Apesar de, muitas vezes, se utilizar a palavra exatidão no mundo da engenharia, sabe-se que, em muitos casos, trata-se de um eufemismo. A arte da engenharia consiste em projetar, construir, implementar e gerenciar diferentes tipos de sistemas que podem trazer consequências tanto positivas como negativas para os trabalhadores, para a sociedade, para o meio ambiente. Aconteceu um evento ou uma sequência de eventos que levou ao rompimento. A engenharia de barragens de rejeitos conhece e é capaz de controlar todos os eventos passiveis de acontecer e que, articulados, podem levar ao rompimento? Há dois caminhos: primeiro, o conhecimento total; a engenharia conheceria tudo e seria capaz de projetar de modo a evitar, com certeza, todos os eventos nocivos. Segundo: a engenharia não conhece a totalidade dos fenômenos, mas sabe que, fazendo como sempre se fez (com o conhecimento empírico) e com margens de segurança grandes, a probabilidade de rompimento se aproxima de zero. Ninguém projeta uma barragem para que ela se rompa. Mas se projeta uma barragem sem dominar na totalidade os eventos que podem ocorrer. Quando se conhece a totalidade da situação, o que deve ser feito para evitar o rompimento é 100% conhecido ­ e precificado. Entretanto, isso nunca acontece, a probabilidade de ocorrência de eventos nunca é completamente conhecida, eles não são determinísticos e a incerteza é um fato.

Will it be possible, sometime in the future, to exactly explain what happened in the dam failure in Brumadinho? Although word "exactness" is often used within the world of engineering, it is often known to be an euphemism. The engineering art consists in projecting, building, implementing and managing different types of systems which might have both positive and negative consequences for workers, society and the environment. Some event or series of events culminated in the dam failure. Is tailings dam engineering aware of and able to control all possible events which together might cause a failure? There are two possible paths: one involves absolute knowledge ­ engineering has absolute knowledge of everything and is able to design projects in a way to avoid any harmful event. According to the other, while engineering does not have absolute knowledge of all the phenomena, its traditional know-how (empirical knowledge) and wide margins of safety make the odds of dam failure come close to zero. No one projects a dam just to fail. But dams are projected without absolute control of all possible events. When the entire situation is known, all that should be done to avoid failures is 100% known ­ and the price fixed. However, this never happens, the probability of the occurence of events are never completely known, they are not deterministic and uncertainty is always a fact.

Brumadinho: between prudence and probability, tragedy.

Will it be possible, sometime in the future, to exactly explain what happened in the dam failure in Brumadinho? Although word "exactness" is often used within the world of engineering, it is often known to be an euphemism. The engineering art consists in projecting, building, implementing and managing different types of systems which might have both positive and negative consequences for workers, society and the environment. Some event or series of events culminated in the dam failure. Is tailings dam engineering aware of and able to control all possible events which together might cause a failure? There are two possible paths: one involves absolute knowledge - engineering has absolute knowledge of everything and is able to design projects in a way to avoid any harmful event. According to the other, while engineering does not have absolute knowledge of all the phenomena, its traditional know-how (empirical knowledge) and wide margins of safety make the odds of dam failure come close to zero. No one projects a dam just to fail. But dams are projected without absolute control of all possible events. When the entire situation is known, all that should be done to avoid failures is 100% known - and the price fixed. However, this never happens, the probability of the occurence of events are never completely known, they are not deterministic and uncertainty is always a fact.

Será possível, em algum momento no futuro, explicar com exatidão o que aconteceu no caso do rompimento da barragem de Brumadinho? Apesar de, muitas vezes, se utilizar a palavra exatidão no mundo da engenharia, sabe-se que, em muitos casos, trata-se de um eufemismo. A arte da engenharia consiste em projetar, construir, implementar e gerenciar diferentes tipos de sistemas que podem trazer consequências tanto positivas como negativas para os trabalhadores, para a sociedade, para o meio ambiente. Aconteceu um evento ou uma sequência de eventos que levou ao rompimento. A engenharia de barragens de rejeitos conhece e é capaz de controlar todos os eventos passiveis de acontecer e que, articulados, podem levar ao rompimento? Há dois caminhos: primeiro, o conhecimento total; a engenharia conheceria tudo e seria capaz de projetar de modo a evitar, com certeza, todos os eventos nocivos. Segundo: a engenharia não conhece a totalidade dos fenômenos, mas sabe que, fazendo como sempre se fez (com o conhecimento empírico) e com margens de segurança grandes, a probabilidade de rompimento se aproxima de zero. Ninguém projeta uma barragem para que ela se rompa. Mas se projeta uma barragem sem dominar na totalidade os eventos que podem ocorrer. Quando se conhece a totalidade da situação, o que deve ser feito para evitar o rompimento é 100% conhecido ­ e precificado. Entretanto, isso nunca acontece, a probabilidade de ocorrência de eventos nunca é completamente conhecida, eles não são determinísticos e a incerteza é um fato.

Impact of a multidisciplinary team for the management of thrombotic microangiopathy.

BACKGROUND: Thrombotic microangiopathy (TMA) is an important complication associated with several diseases that are rare and life-threatening. TMA is common to thrombotic thrombocytopenic purpura (TTP) and hemolytic uremic syndrome (HUS). TTP is defined by a severe deficiency of ADAMTS13, and early treatment is associated with good prognosis. The diagnosis of HUS can be difficult due to the potential multiple etiologies, and the best treatment option in most cases is not well-established yet. The implementation of a multidisciplinary team (MDT) could decrease the time to diagnosis and treatment for HUS and may improve the outcomes of these patients. OBJECTIVE: To determine the impact of MDT on morbidity and mortality [death or chronic renal replacement therapy (CRRT) requirements], incidence and response time [(RT) defined as the period between hospital admission and the first day of direct therapy administration], length of stay at an intensive care unit (ICU-LOS) and total hospitalization (T-LOS) were also assessed. METHODS: We compared a pre-MDT implementation period (from January/2008 to May/2016) versus post-MDT period (from May/2016 to December/2016). The screening TMA diagnosis was made according the following criteria: hemolytic anemia, thrombocytopenia and acute renal damage and without ADAMTS13 deficiency. An online chat was implemented to provide instant medical information. RESULTS: Twenty-eight patients were included. The incidence changed from 2.3 cases/pre-MDT: (all cases: n = 18) to 10 cases/year post-MDT (all cases: n = 10). Two patients died in pre-MDT and post- MDT (11% versus 20%, P = 0.60). From pre-MDT, the number of patients who required CRRT by post-MDT decreased from 7 (39%) to 0, P = 0.03. Similarly, RT, ICU-LOS and T-LOS [median(p25-p75)] decreased from 10 (2-12) days to 0.5 (0-1.5) days, P = 0.04, from 16 (9-30) days to 10 (4-13) days, P = 0.01 and from 33 (22-53) days to 16 (12-32) days, P < 0.01, respectively. CONCLUSION: MDT implementation was associated with a greater number of patients who meet TMA criteria. A decrease in the RT and T-LOS periods were observed and associated with better outcomes in these patients.

Curiosidade, prazer e transgressão: pilares motivadores do consumo de crack / Curiosity, pleasure, transgression: pillars of the motivation to consume crack / Curiosidad, placer y transgresión: los pilares motivadores del consumo de crack

Estudo descritivo, com abordagem qualitativa, cujos objetivos foram descrever as motivações que levaram os usuários a experimentarem o crack e analisar as repercussões psicossociais decorrentes dos comportamentos assumidos para manutenção do consumo. Os entrevistados foram dez dependentes de crack, sendo nove do sexo masculino e um do feminino, em um Centro de Atenção Psicossocial Álcool e Drogas (CAPS AD), localizado no Município de Duque de Caxias, na Baixada Fluminense, no Estado do Rio de Janeiro, em 2011. Os resultados evidenciaram que a curiosidade e o prazer foram decisivos na iniciação do uso do crack. As transgressões referidas nas mais variadas formas de violência caracterizam o comportamento dos usuários e a venda do corpo como forma de renda para o consumo da droga. Conclui-se que o abuso de crack pode ser um artifício capaz de transformar uma vida marcada pela falta, discriminação e ausência de perspectivas, em uma busca constante por prazer

This qualitative, descriptive study described the motivations that led users to try crack, and analyzed the psychosocial effects of the behavior pursued to maintain consumption. The interviewees were ten crack addicts, nine male and one female, at an alcohol and drug psychosocial care center (Centro de Atenção Psicossocial Álcool e Drogas, CAPS AD) in Duque de Caxias municipality, in the Baixada Fluminense, Rio de Janeiro State, in 2011. The results showed that curiosity and pleasure were decisive in their starting to use crack. User behavior was characterized by transgressions reported in various forms of violence and the sale of the body as a source of income to support drug use. It was concluded that crack abuse can be a device for transforming a life marked by failure, discrimination and lack of prospects into a constant search for pleasure.

Estudio descriptivo con enfoque cualitativo que tuvo como objetivos describir las motivaciones que llevaron a los usuarios a probar el crack y analizar los efectos psicosociales derivados de la conducta asumida para mantener el consumo. Los entrevistados fueron 10 adictos al crack, nueve hombres y una mujer, en un Centro de Atención Psicosocial Alcohol y Drogas (CAPS AD), situado en el municipio de Duque de Caxias , en la Baixada Fluminense, en el Estado de Río de Janeiro en 2011. Los resultados mostraron que la curiosidad y el placer fueron decisivos en el inicio del consumo de crack. Los delitos mencionados en las más variadas formas de violencia caracterizan la conducta de los usuarios caracterizan la violencia y la venta del propio cuerpo como medio de ingresos para el consumo de la droga. Se concluye que el abuso de crack puede ser un dispositivo capaz de transformar una vida marcada por la falta, la discriminación y la ausencia de perspectivas, en una constante búsqueda de placer.

Acute lymphoblastic leukemia with e1a3 BCR/ABL fusion protein. A report of two cases.

B Acute Lymphoblastic leukemia (B-ALL) with Philadelphia chromosome (Ph') is a neoplasm of lymphoblast committed to the B cell lineage. The clinical presentation of B-ALL Ph'+ is similar to B-ALL, but is more common in adults than in children. The e1a3 rare variant is produced by the fusion of BCR exon 1 to ABL exon 3. The presence of this translocation has been associated with good disease outcome for chronic myeloid leukemia in a very small series of only 5 cases; there is no such evidence for B-ALL. We report two new cases of B-ALL Ph+ with the rare e1a3 fusion transcript. The e1a3 and e1a2 (p190) transcripts have been reported to have a similar molecular weight and probably a similar clinical profile, thus in these cases the presence of e1a3 was associated with extramedullary infiltration and disease acceleration.

Derrame pleural leucémico: aproximación diagnóstica y controversias en pleurodesis / Leukemic Pleural Effusion: Diagnostic Approach and Controversies in Pleurodesis

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