Uso do Mini-Clinical Evaluation Exercise (Mini-CEX) na residência médica: uma revisão de escopo / Use of Mini-Clinical Evaluation Exercise (Mini-CEX) in medical residency: a scoping review

Resumo Introdução: Com a evolução do ensino médico para currículos baseados em competências, fez-se necessária uma readequação dos currículos e dos métodos de avaliação, com maior enfoque sobre o cenário de prática profissional e, portanto, na utilização de ferramentas como o Mini-Clinical Evaluation Exercise (Mini-CEX). Objetivo: Este estudo teve como objetivo avaliar o uso da estratégia Mini-CEX como método de avaliação nos programas de residência médica. Método: Trata-se de uma revisão de escopo, cuja estratégia de busca realizada no PubMed resultou em 578 artigos. Após aplicar a metodologia do Instituto Joanna Briggs para inclusão e exclusão, foram selecionados 24 estudos transversais. Resultado: Selecionaram-se artigos referentes a estudos realizados entre 1995 e 2021, em diversos continentes, diferentes programas de residência, e cenários ambulatorial, internação e de emergência. O Mini-CEX mostrou-se aplicável no contexto da residência médica, pois trata-se de uma avaliação observacional direta do atendimento realizado pelo médico residente nos diversos cenários de atuação, como ambulatórios, internações e emergências. Trata-se de uma avaliação com tempo de observação variando de dez a 40 minutos e que permite a abordagem de vários aspectos do atendimento médico, como anamnese, exame físico, raciocínio clínico e aconselhamento, além de possibilitar a realização de um feedback sobre o desempenho dos residentes. Conclusão: O Mini-CEX constitui uma ferramenta de fácil aplicabilidade e promove alto grau de satisfação dos envolvidos, podendo ser utilizada de forma rotineira nos programas de residência médica.

Abstract Introduction: With the evolution of medical education towards competency-based curriculum, the need has emerged to reconfigure curriculum and assessment methods, with increased focus on the professional practice setting, thus leading to the utilization of tools such as the mini-CEX (mini-Clinical Evaluation Exam). Objective: To evaluate the use of the mini-CEX strategy as an assessment method in medical residency programs. Method: This is a scoping review, and the search performed on PubMed resulted in 578 articles. After applying the Joanna Briggs Institute methodology for inclusion and exclusion, 24 cross-sectional studies were selected. Results: The selected articles were based on studies conducted between 1995 and 2021, in various continents and in both clinical and surgical residency programs, including outpatient, inpatient, and emergency settings. The Mini-CEX was shown to be applicable in the context of medical residency, as it is an observational assessment of the care provided by the resident physician in various practice settings such as outpatient clinics, inpatient wards, and emergency departments. It involves a variable observation time ranging from 10 to 40 minutes and allows for the evaluation of various aspects of medical care, including history taking, physical examination, clinical reasoning, counseling, and provides an opportunity for providing feedback on the residents' performance. Conclusion: The mini-CEX is a tool that is easy to implement and promotes a high degree of satisfaction among stakeholders. It could be used more routinely in medical residency programs.

Extracorporeal photopheresis in chronic graft-versus-host disease: clinical description and economic study

Abstract Introduction Although extracorporeal photopheresis (ECP) is a promising second-line therapy in the treatment of chronic graft-versus-host disease (cGVHD), its use is limited by its high cost. This study aims to describe the clinical evolution of patients who underwent ECP therapy for cGVHD and to perform an economic analysis of the therapy Methods This was a case series between 2016 and 2020 describing the clinical response to ECP and a micro-cost analysis of the therapy using time-driven activity-based costing. Results Six patients underwent ECP for corticosteroid-dependent cGVHD The cost per ECP session is 14,960.90 Brazilian reais (BRL), which primarily consists of the ECP kit with an activator (82.78%), followed by the hospital's physical structure (14.66%), human resources (2.48%) and exams/inputs (0.08%). The number of sessions performed ranged from 2 to 42. The total cost of the therapy per patient ranged from BRL 30,000 to 500,000. Conclusion The response of the patient with cGVHD to treatment with ECP was variable. These micro-costing results can be used to develop remuneration and cost control strategies in hematopoietic stem cell transplantation programs, as well as in further economic studies.

Investigation of oral and general health status and IL-1β gene polymorphism as risk factors for oral mucositis in hematopoietic stem cell transplantation patients

Abstract: The aim of the present study was to analyze the relationship of OM with possible risk factors such as oral health condition, immunological status and IL-1β profile in patients submitted to hematopoietic stem cell transplantation (HSCT). Fifty-four individuals submitted to HSCT were included. All patients received previous dental treatment and photobiomodulation (PBM) as the institutional OM preventive protocol. OM scores, immune status, and IL-1β levels were determined during the conditioning period and at D+3 and D+8 after HSC infusion. IL-1β gene polymorphism was also analyzed during conditioning. Possible associations of OM with risk factors were analyzed using conditional Fisher's exact test. OM was observed in 34 patients (62.9%) classified as Grade 1 (13 patients/24.1%), Grade 2 (14 patients/25.9%), Grade 3 (3 patients/5.5%), and Grade 4 (4 patients/7.4%). Allogeneic HSCT individuals exhibited a higher OM grade than autologous subjects. Moreover, an association was observed between severe OM and severe gingivitis (p = 0.01), neutropenia (p = 0.03), and leukopenia (p = 0.04). A significant association between OM and lower IL-1β levels was detected at three time points, i.e., conditioning (p = 0.048), D+3 (p = 0.01), and D+8 (p = 0.005). The results showed that IL-1β gene polymorphism was not associated with OM. Our study provided important insights into the scope of OM risk factors in the setting of HSCT. Patients submitted to HSCT with severe gingivitis prior to chemotherapy and with severe neutropenia and leukopenia exhibited a higher OM grade. Further investigation will be necessary to better understand the exact role of IL-1β in the context of OM pathobiology and to validate cytokine analysis in larger cohorts.

Malignant pleural effusion in multiple myeloma: a case report / Derrame pleural malign em mieloma múltiplo: relato de caso

Multiple myeloma (MM) is a malignant neoplasm of monoclonal plasma cells that accumulate in bone marrow (BM). Malignant pleural effusions (MPE), as part of multiple myeloma clinical presentation, are unusual. Is even more rare as the first sign of presentation, occurring in less than 1% of the cases. The most common associated immunoglobulin with malignant pleural effusions is IgA subtype (80%). This condition carry a poor prognosis. We aim to describe a refractory case of multiple myeloma with extensive disease that presented with extramedullary relapse with malignant pleural effusions , besides discussing the importance of differential diagnosis.

O mieloma múltiplo (MM) é uma neoplasia maligna de células plasmáticas monoclonais que se acumulam na medula óssea (MO). Os derrames pleurais malignos (EPM), como parte da apresentação clínica do mieloma múltiplo, são incomuns. É ainda mais raro como primeiro sinal de apresentação, ocorrendo em menos de 1% dos casos. A imunoglobulina associada mais comum a derrames pleurais malignos é o subtipo IgA (80%). Esta condição carrega um mau prognóstico. Nosso objetivo é descrever um caso refratário de mieloma múltiplo com doença extensa que apresentou recidiva extramedular com derrame pleural maligno, além de discutir a importância do diagnóstico diferencial

Donor characteristics and hematopoietic stem cell transplantation outcome: experience of a single center in Southern Brazil

ABSTRACT Background: Hematopoietic stem cell transplantation is a curative treatment for many patients with hematological disorders. Donor-recipient genetic disparity, especially involving the human leukocyte antigen system is a critical factor for transplant outcome. Objective: To evaluate retrospectively donor characteristics and correlations with the occurrence of acute and chronic graft-versus-host disease, disease-free survival and overall survival in a Brazilian population submitted to allogeneic hematopoietic stem cell transplantation between 1994 and 2012 in a single center. Results: Three hundred and forty-seven consecutive transplantations were included. Related transplants (81.2%) were significantly more common than unrelated transplants (18.7%); donor and recipient median ages were 34 (range: 1-61) and 33 (range: 3-65) years respectively with donor HLAs being matched for 333 (95.9%) patients. Donor gender, cytomegalovirus status and ABO incompatibility did not influence the five-year overall survival. In univariate analyses, overall survival was negatively influenced by the presence of acute graft-versus-host disease (33% vs. 47%, respectively; p-value = 0.04), unrelated transplant (41.5% vs. 50.9%, respectively; p-value = 0.045) and donors aged over 40 years (41% vs. 52%, respectively; p-value = 0.03). Older donors were associated with a higher rate of acute (52% vs. 65.8%; p-value = 0.03) and chronic graft-versus-host disease (60% vs. 43%, respectively; p-value = 0.015). In multivariate analyses, acute graft-versus-host disease [relative risk (RR): 1.8; 95% confidence interval (CI): 1.1-29; p-value = 0.008] and older donors (RR: 1.6; 95% CI 1.11-2.24; p-value = 0.013) were associated with higher transplant-related mortality. Conclusions: In transplant patients, to have a donor older than 40 years of age seems to significantly increase the incidence of acute and chronic graft-versus-host disease and transplant-related mortality with no impact on disease-free survival and overall survival. In spite of the rather small cohort of patients, these findings are similar to what is described in the literature suggesting that a younger donor should be chosen whenever possible.

Quantification of mixed chimerism allows early therapeutic interventions

Hematopoietic stem cell transplantation is the curative option for patients with myelodysplastic syndrome; however, it requires a long post-transplantation follow-up. A 53-year-old woman with a diagnosis of myelodysplastic syndrome underwent related donor allogeneic hematopoietic stem cell transplantation in July 2006. Three months after transplantation, a comparative short tandem repeat analysis between donor and recipient revealed full chimerism, indicating complete, healthy bone marrow reconstitution. Three years and ten months after hematopoietic stem cell transplantation, the patient developed leukopenia and thrombocytopenia. Another short tandem repeat analysis was carried out which showed mixed chimerism (52.62%), indicating relapsed disease. A donor lymphocyte infusion was administered. The purpose of donor lymphocyte infusion is to induce a graft-versus-leukemia effect; in fact, this donor's lymphocyte infusion induced full chimerism. Successive short tandem repeat analyses were performed as part of post-transplantation follow-up, and in July 2010, one such analysis again showed mixed chimerism (64.25%). Based on this finding, a second donor lymphocyte infusion was administered, but failed to eradicate the disease. In September 2011, the patient presented with relapsed disease, and a second related donor allogeneic hematopoietic stem cell transplantation was performed. Subsequent short tandem repeat analyses revealed full chimerism, indicating complete bone marrow reconstitution. We conclude that quantitative detection of mixed chimerism is an important diagnostic tool that can guide early therapeutic intervention...

Fibrobroncoscopia no diagnóstico e decisão terapêutica de infecções respiratórias em pacientes neutropênicos febris hematológicos / Fiberoptic bronchoscopy in the diagnosis and therapeutic decision for respiratory infections in hematological febrile neutropenic patients

INTRODUÇÃO: A neutropenia febril é uma complicação frequente dos pacientes submetidos ao tratamento quimioterápico ou Transplante de Célula Tronco Hematopoiética (TCTH). A fibrobroncoscopia (FBC) flexível tem sido utilizada para auxiliar no diagnóstico de doenças pulmonares. No entanto, não há consenso em relação ao benefício do exame para estabelecer diagnóstico e alterar o tratamento das doenças pulmonares nesse contexto. Estudos prévios, retrospectivos e bastante heterogêneos, com pacientes imunocomprometidos não-HIV mostraram que o rendimento da fibrobroncoscopia para estabelecer diagnóstico etiológico varia de 13 a 81% e gera alteração de terapêutica em 5 e 51%. O objetivo deste estudo foi avaliar o rendimento da Fibrobroncoscopia, o risco ao procedimento em pacientes hematológicos e neutropênicos. MÉTODOS: Estudo transversal retrospectivo que avaliou pacientes com neoplasia hematológica e neutropenia febril e que tenham sido submetidos à fibrobroncoscopia diagnóstica entre janeiro de 2011 e dezembro de 2012 internados no Hospital de Clínicas de Porto Alegre. RESULTADOS: Foram incluídos 45 pacientes: 18 (36%) tiveram resultado positivo no Lavado Broncoalveolar (LAB), sendo que houve mudança na conduta terapêutica em 95% dos pacientes que apresentaram positividade no LAB. Com relação ao risco do procedimento tivemos uma taxa de 2,2% de complicação, com um paciente que apresentou dessaturação imediatamente após o procedimento. CONCLUSÃO: Apesar do número limitado de pacientes, nossos achados indicam que a realização da fibrobroncoscopia com LAB em pacientes neutropênicos é segura e com um rendimento semelhante aos descritos na literatura

INTRODUCTION: Febrile neutropenia is a common complication in patients undergoing chemotherapy or hematopoietic Stem Cell Transplantation (HSCT). Flexible fiberoptic bronchoscopy has been used to aid in the diagnosis of pulmonary diseases. However, there is no consensus regarding the benefit of the exam in establishing diagnosis and in changing the treatment of lung disease in this context. Previous retrospective studies, quite heterogeneous and with non-HIV immunocompromised patients, showed that the yield of fiberoptic bronchoscopy in establishing etiology ranges from 13% to 81%, and in changing therapy, from 5% to 51%. To evaluate the efficiency of Fiberoptic bronchoscopy and the procedure-related risk for neutropenic patients with hematologic malignancy. METHODS: This retrospective cross-sectional study analyzed the medical records of patients with hematologic malignancy with febrile neutropenia who had undergone diagnostic fiberoptic bronchoscopy between January 2011 and December 2012 at the Hospital de Clínicas de Porto Alegre. RESULTS: A total of 45 patients were included: 18 (36%) tested positive for bronchoalveolar lavage, with change in therapeutic management occurring for 95% of them. The procedure-related risk was 2.2%, with one patient showing desaturation immediately after the procedure. CONCLUSION: Despite the limited number of patients, our findings indicate that fiberoptic bronchoscopy in neutropenic patients is safe, and the results are similar to those previously reported

Clinical impact of systematic nutritional care in adults submitted to allogeneic hematopoietic stem cell transplantation

BACKGROUND: The development of nutrition care programs for patients undergoing hematopoietic stem cell transplantation is necessity in view of the rapid and aggressive consequences frequently seen with this procedure. Patients require constant care to reduce complications and to contribute to the success of therapy. METHODS: In an attempt to ascertain the impact of systematic nutritional care on patients submitted to allogeneic hematopoietic stem cell transplantation, the present study assessed the nutritional and clinical status, use of parenteral nutrition, and complication and mortality rates in two groups of patients, who were submitted to transplantation between April 2003 and December 2004 (Non-intervention Group - NIG; n = 57) and between March 2006 and January 2008 (Intervention Group - IG; n = 34). RESULTS: There were no significant differences between groups in terms of clinical or nutritional profiles. Additionally, the length of hospital stay and complication and mortality rates were similar for both groups. However, time on parenteral nutrition during treatment was shorter for the IG [median 6.5 days (range: 1-28) for related donor recipients and 11 days (range: 1-21) for unrelated donor recipients] than for the NIG [median 20.5 days (range, 4-73) for patients submitted to myeloablative conditioning and 18.5 days (range: 11-59 days) for those submitted to nonablative conditioning]. CONCLUSION: The implementation of a nutritional follow-up and therapy protocol for adult patients submitted to hematopoietic stem cell transplantation shortens the duration of parenteral nutrition. It certainly has an impact on hospitalization costs and, potentially, on the rate of complications, even though this was not demonstrated in this study.

Transplante de células-tronco hematopoéticas em linfoma Hodgkin / Stem cell transplantation in Hodgkin lymphoma

O linfoma Hodgkin(LH) é uma malignidade hematológica que conta com um armamentário terapêutico selecionado de acordo com o estadiamento e a classificação prognóstica de cada doente. A sobrevida dos pacientes tratados para o LH clássico vem aumentando significativamente, com taxas de cura entre 80 por cento-85 por cento. Entretanto, 20 por cento-25 por cento são refratários aos tratamentos iniciais e cerca de 30 por cento recaem após ter alcançado resposta completa. Os pacientes considerados com falha à terapia de primeira linha ainda têm uma segunda chance de cura se apresentarem quimiossensibilidade aos esquemas de salvamento, seguido por uma das modalidades de transplante de células-tronco hematopoéticas (TCTH). O TCTH autólogo representa uma estratégia atrativa para os pacientes com LH que falham ao tratamento convencional de primeira linha. Os resultados em termos de sobrevidas livre de doença e global são superiores aos esquemas de salvamento com quimioterapia convencional. Este procedimento tem finalidade curativa para 50 por cento dos pacientes em segunda remissão quimiossensíveis e pode levar a remissões duráveis naqueles com mais de duas linhas de terapia. Atualmente, o TCTH alogênico, basicamente com condicionamento de intensidade reduzida (RIC), está indicado em pacientes com recaída precoce após o TCTH autólogo ou em pacientes bastante jovens com refratariedade a mais de duas linhas de tratamento convencional.

Hodgkin's Lymphoma is a hematologic malignancy with a wide range of therapeutic options that must be chosen according to the stage and the prognostic classification of each patient. The overall survival of patients treated for classic Hodgkin's Lymphoma is increasing significantly, with current cure rates being between 80 percent and 85 percent. Nevertheless, 20 percent to 25 percent are refractory to the initial treatment and about 30 percent relapse after having reached a complete response. Patients that have failed standard therapy still have a second chance of cure if they present chemosensitivity to cure schemes, followed by one type of hematopoietic stem cell transplantation (TCTH). Autologous TCTH is an attractive strategy for Hodgkin's Lymphoma patients that fail in the conventional standard therapy. The results in terms of overall survival and disease-free survival are higher than the cure schemes with conventional chemotherapy. This procedure addresses the cure in 50 percent of chemosensitive patients in second remission, and can lead to lasting remissions for those with more than two lines of treatment. Today, allogeneic TCTH, basically with reduced intensity conditioning (RIC) is indicated for patients with premature relapse after autologous TCTH or for young patients refractory to one or more lines of conventional treatment.

Rhinosinusitis in autologous and allogeneic bone marrow transplantation: a retrospective study on the performance of imaging studies on severity and prognostic evaluation / Rinossinusite em transplante de células-tronco hematopoéticas autólogo e alogênico: um estudo retrospectivo sobre o desempenho de estudos de imagem na avaliação de severidade e prognóstico

The objective of this work was to evaluate the diagnostic and prognostic performance of a traditional imaging staging system for rhinosinusitis in the bone marrow transplantation (BMT) scenario. A retrospective cohort study was carried out at a bone marrow transplantation referral center involving subjects who underwent allogeneic or autologous BMT from September 1st 2005 to September 31st 2007 and later evolved with rhinosinusitis during the BMT inpatient period. Patients who had a previous history of sinusal disease or otolaryngologic surgery were excluded from the study. Data concerning mortality, the treatment of rhinosinusitis and BMT outcomes were extracted from medical files. The collected parameters were compared to the Lund-Mackay tomographic staging system score which was calculated based on available tomography films of each patient. A total of 85 BMT were performed and 23 allogeneic and 14 autologous (43.5 percent) BMT patients evolved with rhinosinusitis during transplantation. A significant association with LMS was found for the absolute neutrophil count (ANC), with a higher ANC (>500/mm3) correlating with a higher LMS (Mean LMS for lower ANC 6.08 and higher ANC 9.71 points, p<0.05). Need for surgical management and post-BMT admissions, the resolution of the rhinosinusitis and overall mortality had no significant correlation with LMS. Patients with less than 500 neutrophils/mm3 are known to be prone to more severe infections, but paradoxically showed lower LMS when developing rhinosinusitis. However, there were no differences in the main outcomes between those with higher and lower LMS. This would possibly lead to an equivocal assumption of a less severe disease. Severely neutropenic patients are probably not able to mount an effective inflammatory response capable of inducing significant tomographic abnormalities. So, this imaging study would not be able to adequately evaluate the extent of sinusal involvement. We thus ...

O objetivo deste trabalho foi avaliar o desempenho diagnóstico e prognóstico de um escore de estadiamento de rinossinusite (RS) por tomografia em pacientes submetidos a transplante de medula óssea (TMO). Realizou-se um estudo de coorte retrospectivo de pacientes submetidos a transplante de medula óssea (autólogo e alogênico) de 1º de setembro de 2005 a 31 de setembro de 2007 que desenvolveram RS durante o período de internação do transplante. Pacientes com história prévia de doença sinusal ou cirurgia otorrinolaringológica foram excluídos do estudo. Dados relacionados à mortalidade, resolução da RS e desfechos do TMO foram extraídos do prontuário médico. Os parâmetros coletados foram correlacionados com o escore de estadiamento de Lund-Mackay (ELM), que foi calculado com base nas alterações tomográficas de cada paciente. Um total de 85 TMO foram realizados e 37 (23 alogênicos e 14 autólogos) destes pacientes desenvolveram RS durante o transplante. Uma correlação significativa com o ELM foi encontrada quando se considerava a contagem absoluta de neutrófilos (CAN), com uma CAN mais alta (>500/mcl) se associando com um ELM de maior valor (média de escore para CAN baixa 6,08 e CAN alta 9,71 pontos, p<0,05). A necessidade de intervenção cirúrgica e reinternações pós-TMO, resolução da RS e mortalidade geral não mostraram correlação com o ELM. Mesmo assumindo que a neutropenia severa é um fator de risco relevante para intercorrências infecciosas durante o TMO, paradoxalmente, os pacientes com menos de 500 neutrófilos/mcl mostraram um ELM de menor severidade, embora não tenham evoluído de maneira diferente daqueles com maior ELM. É provável que ELM mais alto esteja simplesmente ligado ao fato de uma CAN mais alta levar a uma maior reação inflamatória e consequente alteração tomográfica. Desta forma, o ELM não parece útil na avaliação de pacientes altamente imunossuprimidos como os do TMO. Rev. Bras. Hematol. Hemoter.

Sobrevida de pacientes submetidos a transplante alogênico de medula óssea / Survival of patients submittes to allogeneic boné marrow transplantation

Introdução: O transplante de células-tronco hematopoiéticas (TCTH) alogênico é um procedimento que oferece um potencial de cura para doenças hematológicas malignas e benignas. O benefício da técnica está especialmente relacionado ao aumento da sobrevida em pacientes com doadores HLA-compatíveis em cujos casos o tratamento quimioterápico mostrou-se insuficiente ou ineficaz. Objetivos: Analisar a sobrevida de pacientes que receberam TCTH alogênico aparentado no Serviço de Hematologia Clínica e Transplante de Medula Óssea (SHCTMO) do Hospital de Clínicas de Porto Alegre (HCPA). Métodos: Estudo de coorte prospectiva com análise de sobrevida de pacientes transplantados entre 1994 e 2003. Resultados: Foram analisados 133 pacientes com idade média de 30,8±14,8 anos com um tempo médio de 26,8 meses entre o diagnóstico e o TCTH. Cinco anos após o transplante, 71 pacientes (53,4%) estavam vivos, 22 pacientes tinham leucemia mieloide aguda (LMA), 54, leucemia mieloide crônica (LMC), e seis padeciam de síndrome mielodisplásica (SMD), sendo que, em 5 anos, a sobrevida foi de 52, 50 e 33%, respectivamente. Dos 26 pacientes transplantados por anemia aplásica (AA), 66,7% tinham idade inferior a 20 anos, e 61,5% dos que tinham mais de 20 anos estavam vivos. Conclusão: Embora, no nosso estudo, o tempo médio entre o diagnóstico e o transplante tenha sido superior a 2 anos, e embora nossa análise tenha sido apenas estratificada pelo tipo da doença, independentemente do regime de condicionamento ou da fase da doença no momento do TCTH, nossos resultados são superponíveis aos descritos na literatura mundial.

Background: Hematopoietic stem cell transplantation (HSCT) represents a curative alternative for malignant and benign hematological diseases. The benefits of the technique are especially related to an increase in the survival of patients with HLA-compatible hematopoietic stem cell donors when chemotherapy or clinical therapy has resulted ineffective. Objectives: To analyze the survival of patients submitted to allogeneic HSCT at the Hematology and Bone Marrow Transplant Service of Hospital de Clínicas de Porto Alegre. Methods: A prospective cohort of all patients submitted to transplantation between 1994 and 2003 was analyzed for overall survival. Results: A total of 133 patients were submitted to transplantation in the study period, with a mean age of 30.8±14.8 years; mean time elapsed between diagnosis and transplant was 26.8 months. Five years after the procedure, 71 patients (53.4%) were alive, 22 patients had acute and 54 had chronic myeloid leukemia, and six patients presented myelodysplastic syndrome; the 5 year overall survival was 52, 50, and 33%, respectively. Of the 26 patients transplanted for aplastic anemia, 66.7% had 20 or less years of age, and 61.5% of the patients older than 20 years were alive. Conclusion: Although the mean time elapsed between diagnosis and transplantation was over 2 years and although our results were stratified by type of disease only, the findings herein reported are similar to those found in the literature, independently of conditioning regimen or disease stage at the time of transplant.

Leucemia mielóide aguda: o olhar dos anos 2000 no Serviço de Hematologia do Hospital de Clínicas de Porto Alegre-RS / Acute myeloid leukemia from the year 2000 point of view Hematology Service - Hospital de Clínicas de Porto Alegre-RS

A leucemia mielóide aguda (LMA) representa uma preocupação para os especialistas, porque perfaz um percentual alto das leucemias no adulto e o sucesso terapêutico ainda é insatisfatório. A partir do ano 2000, o Serviço de Hematologia do Hospital de Clínicas de Porto Alegre definiu estratégias para diagnóstico, tratamento e seguimento das LMAs, de acordo com o subtipo FAB, idade, citogenética e performance status (ECOG). Todos os casos de LMA "de novo"não promielocítica, em adultos (15 a 65 anos) foram acompanhados prospectivamente, desde outubro de 2001, data da implantação do protocolo c,ompreendendo três fases de tratamento: indução com o tradicional "7+3", citarabina 100 mg/m²/dia em infusão contínua em 7d, e daunorrubicina 60 mg/m²/dia em 3d e citarabina intratecal no D1 nas LMA M4 e M5. Após a recuperação medular, segue a consolidação idêntica à indução e posteriormente a intensificação com dois ou três ciclos de altas doses de citarabina 6 g/m²/dia por três dias. Foram diagnosticados, entre outubro/01 e dezembro/05, 69 pacientes portadores de LMA e destes, 39 com LMA "de novo"e idade entre 15 e 65 anos. Neste grupo foram analisadas a taxa de remissão, a taxa de recaída, a refratariedade e o tempo de sobrevida global. No final da observação foram encontrados: a taxa de indução de remissão 75 por cento; aconteceram 12 (40 por cento) recaídas, 7 (19 por cento) foram refratários ao tratamento. A sobrevida global foi 37 por cento em 56 meses, representando um incremento aos resultados obtidos no Serviço na década passada.

Acute myeloid leukemia (AML) is still a concern for hematologists as it represents a significant percentage of adult leukemias and the therapeutic success rates are unsatisfactory. In 2000, the Hematology Department of Hospital de Clínicas de Porto Alegre defined strategies for the diagnosis, treatment and follow up of AML patients according to the FAB subtype classification, age, cytogenetic tests and performance status (ECOG). Patients with promyelocytic leukemia are treated using the AIDA (GIMEMA) protocol with those older than 65 years receiving palliative therapy using hydroxyurea, oral etoposide, thalidomide, subcutaneous cytarabine or an association of drugs. Since October 2001 all our "de novo"AML patients aged 15 to 65 years with non-promyelocytic acute leukemia were prospectively followed up. At diagnosis we start a three phase treatment protocol: induction with a classical "7+3"therapy regimen, that is continuous infusion of 100 mg/m²/day cytarabine for 7 days, 60 mg/m²/day daunorubicin for 3 days and on day 1 an intrathecal cytarabine in AML M4 and M5 cases. After bone marrow recovery, if complete remission is achieved, follow ups involve an identical "7+3"consolidation phase followed by two or three high dose cycles of 6 g/m²/day cytarabine for 3 days. A group of 39 patients diagnosed between October 2001 and December 2005 was followed up until June 2006. Our objectives were to evaluate the effectiveness of the protocol for remission, relapse rates and overall survival. The rate of complete remission was 75 percent. Relapse occurred in 12/29 (40 percent) patients and the overall survival rate at 56 months was 37 percent, showing an improvement on our results of previous decades.

Talidomida e mieloma múltiplo: verificação dos efeitos terapêuticos através de parâmetros clínico e laboratoriais / Thalidomide and multiple myeloma: therapy evaluation using clinical and laboratorial parameters

Nas duas últimas décadas, houve uma mudança radical na terapia e na evolução do mieloma múltiplo(MM), neoplasia hematológica ainda considerada fatal. As pesquisas e investimentos em medicamentos que interferem com a fisiopatogenia e com o microambiente medular estão permitindo o controle e a regressão do clone plasmocitário maligno, mudando as perspectivas da doença. A idéia nova de usar uma droga velha, a talidomida, tem-se mostrado efetiva no MM. Em 1997, apostando nos efeitos imunomoduladores e antiangiogênicos da talidomida, foram iniciados ensaios clínicos para MM refratários. A partir daí, outras ações sobre o plasmócito e microambiente medular foram eficazes contra a doença, não somente em refratários ou recaídos, mas também como terapia de indução e/ou de manutenção da remissão. No Serviço de Hematologia do Hospital de Clínicas de Porto Alegre foram acompanhados 35 portadores de mieloma múltiplo, em uso de doses baixas (100 mg) de talidomida, pelas indicações: 13 - manutenção pós-TMO, 11 - pós-indução, 5 - recaída, 4 - refratariedade e 2 - terapia de indução. O estudo vigorou entre março/01 a dez/03. Os parâmetros avaliados foram: nível Hb, pico da imunoglobulina sérica ou urinária e o número de plasmócitos na medula óssea. As medidas foram tomadas pré-talidomida e após 3, 6 e 12 meses. A taxa de imunoglobulina foi o padrão ouro para avaliação de resposta. Os resultados: a dose terapêutica tolerada em 48 por cento dos pacientes foi 100 mg; 65 por cento dos tratados para induzir remissão (11 pacientes) apresentaram melhora entre 25 por cento-50 por cento no nível da imunoglobulina sérica; 87,5 por cento daqueles que usaram para manutenção de remissão (13 pós-TMO/ 11 pós-indução) mantiveram o mesmo plateau inicial.

Over the last two decades, we have seen a radical change intherapy and progression of multiple myeloma, a malignanthematologic disease that is still considered fatal. Recentinvestment and research on mechanisms that interfere in thephysiopathogenesis and bone marrow microenvironment areturning control and regression of the malignant plasma cellclone into something achievable, which may change expectationsrelated to this disease. The new idea of using an old drug,thalidomide, has shown to be effective in multiple myeloma. In1997, using the known effects of immunomodulation and antiangiogenesisof this drug, clinical trials were started in patients with unresponsive disease. Other therapeutic interventions inthe bone marrow microenvironment and plasma cells have beenadded and proved to be efficacious, not only as a therapy forrefractory patients, but also for induction and/or remissionmaintenance therapy. Thirty-five patients with multiple myelomawere treated with low-dose thalidomide (100 mg) and followedup. .... The study tookplace in the Hematology and Bone Marrow Transplantationservice of the Hospital de Clínicas de Porto Alegre, from March2001 to December 2003. Hemoglobin levels, serum or urineimmunoglobulin peaks and bone marrow plasma cell countswere evaluated. These parameters were assessed before startingwith the drug and after 3.6 and 12 months of usage. Theimmunoglobulin level was considered the gold standard toevaluate the response. The results showed that 100 mg was thetolerable dose for 51% of the patients. Sixty-five percent of thosewho used thalidomide for induction therapy showed a 25 to 50%improvement in immunoglobulin serum levels and 90% of thepatients on maintenance therapy (13 after bone marrowtransplantation, 11 after induction), sustained the sameimmunoglobulin levels of the initial plateau.