Superiority of the triple combination of bortezomib, cyclophosphamide and dexamethasone versus cyclophosphamide, thalidomide and dexamethasone in patients with newly diagnosed multiple myeloma, eligible for transplantation

ABSTRACT Background: The treatment of multiple myeloma (MM) has evolved significantly in the past decade, and new drug combinations have improved the response rates and prolonged survival. Studies comparing different induction chemotherapy regimens have shown that triple combinations have better results than double combinations. However, comparisons among different triple combinations are rare in the literature. Methods: We retrospectively compared two triple combinations comprising bortezomib, cyclophosphamide and dexamethasone (VCD) versus thalidomide, cyclophosphamide and dexamethasone (CTD), and aimed at identifying which of the two combinations would yield better response rates following four induction cycles prior to hematopoietic cell transplantation in patients with untreated multiple myeloma. Results: We retrospectively reviewed the medical records of 311 patients from 24 different centers.The VCD regimen was used as induction therapy by 117 (37.6%) patients, whereas 194 (62.4%) patients received the CTD regimen. After four cycles of induction on an intention-to-treat basis, 54% of the patients in the VCD group achieved at least very good partial response versus 42.8% in the CTD group (p = 0.05). We observed no difference in neuropathy or thrombotic events rates among the two regimens. Conclusion: Our results corroborate the superiority of the triple combination regimes containing bortezomib over the triple combination with thalidomide as pre ASCT induction therapy in MM.

Herpes zoster after autologous hematopoietic stem cell transplantation

ABSTRACT Background: The autologous hematopoietic stem cell transplantation procedure involves immunosuppression of the patient. Thus, the patient has an elevated risk for several diseases, such as infections with the varicella-zoster virus. Prevention protocols have been proposed based on the use of acyclovir from the first day of conditioning, and maintaining this drug for 30-100 days after the procedure or for as much as one year. The objective of this work was to evaluate the incidence of herpes zoster after autologous transplantations related to the early suspension of acyclovir. Methods: A retrospective study was carried out based on the collection of data from 231 medical records of transplant patients in the Bone Marrow Transplant Unit of the teaching hospital of the Universidade Federal de Juiz de Fora in the period between 2004 and 2014. Results: Fourteen (6.1%) patients had herpes zoster in the post-transplant period on average within six months of the procedure. Patients with multiple myeloma (64.3%) were the most affected. There was a statistically significant difference in the age of the patients, with older individuals having a greater chance of developing the infection (p-value = 0.002). There were no significant differences for the other variables analyzed. Conclusion: The early suspension of acyclovir can be safe in patients who receive autologous hematopoietic stem cell transplants. However some groups may benefit from extended prophylaxis with acyclovir, particularly older patients and patients with multiple myeloma.

Transfusion practices in a neonatal intensive care unit in a city in Brazil

OBJECTIVE: Newborn infants are the most heavily transfused population inside intensive care units. The hemoglobin level used to indicate the need of transfusions is not well established. The aim of this study was to evaluate transfusional practices in newborns in the neonatal intensive care units of one specific city. METHODS: Red blood cell transfusion practices of all transfused newborns in all five of the neonatal intensive care units of the city were analyzed. Data are reported as descriptive statistics, including numbers and percentages and means and standard deviation. Univariate analysis, followed by stepwise logistic regression was performed in respect to transfusional data and outcomes. RESULTS: A total of 949 patients were admitted to the intensive care units during the 12-month study period with 20.9% receiving at least one transfusion, most (62.4%) of whom received more than one transfusion. The mean number of transfusions per infant was 2.7 ± 2.16; in the liberal transfusion group the mean number was 1.59 ± 1.63 and in the restrictive group it was 1.08 ± 1.51. The mean hemoglobin and hematocrit levels were 9.0 g/dL (±1.4 g/dL) and 27.4% (±4.3%), respectively. The most common indications for blood transfusions were sepsis and prematurity. CONCLUSION: This study shows that the characteristics and the transfusion practices for newborns admitted in the neonatal intensive care units of Juiz de Fora are similar to recent pubications. There was no significant reduction in the number of transfusions per child in the restrictive group compared to the liberal group. Restrictive transfusions are an independent risk factor for peri-intraventricular hemorrhages and death...

Allogeneic hematopoietic stem cell transplantation in mycosis fungoides / Transplante alogenico de celulas-tronco hematopoieticas em micose fungoide

Mycosis Fungoides is typically an indolent disease in early stages. However, approximately 30% of patients have advanced staged disease at presentation and 20% will develop it at some time. These patients have a poorer prognosis with a median survival of 2-4 years. The only curative option for mycosis fungoides may be hematopoietic allogeneic stem cell transplantation. We report the case of a patient with mycosis fungoides in an advanced stage (IIB), refractory to treatment options. She underwent allogeneic hematopoietic stem-cell transplantation (allo-HSCT). The patient remains in complete remission nineteen months after allo-HSCT. Allogeneic transplantation can alter the natural history of mycosis fungoides and should be considered in patients who have refractory disease or short-lived responses with standard therapies.

Micose Fungoide é tipicamente uma doença indolente em estágios iniciais. No entanto, aproximadamente 30% dos pacientes têm doença avançada na apresentação e 20% irão desenvolvê-la em algum momento. Esses pacientes têm um pior prognóstico com uma sobrevida média de dois a quatro anos. A única possibilidade de cura é o transplante alogênico de células-tronco hematopoiéticas. Relatamos o caso de uma paciente com micose fungoide em estágio avançado (IIB), refratária às opções terapêuticas e que foi submetida a um transplante alogênico de células-tronco hematopoiéticas. A paciente permanece em remissão completa 19 meses após o procedimento. O transplante alogênico é capaz de mudar a história natural da micose fungoide e deve ser considerado em pacientes com doença avançada e refratária aos tratamentos disponíveis.

T-cell large granular lymphocytic leukemia: treatment experience with fludarabine

OBJECTIVES: The aim of this retrospective study was to investigate the results of T-cell large granular lymphocytic leukemia treatment with fludarabine by assessing the complete hematologic response, the complete molecular response, progression-free survival, and overall survival. METHODS: We evaluated the records of six patients with T-cell large granular lymphocytic leukemia who were treated with fludarabine as a first-, second-, or third-line therapy, at a dose of 40 mg/m², for three to five days per month and 6 to 8 cycles. RESULTS: Of the six patients investigated with T-cell large granular lymphocytic leukemia who were treated with fludarabine, five (83.3%) were female, and their median age was 36.5 years (range 18 to 73). The median lymphocyte level was 3.4x10(9)/L (0.5 to 8.9). All patients exhibited a monoclonal T-cell receptor gamma gene rearrangement at diagnosis. Two (33.3%) patients received fludarabine as first-line treatment, two (33.3%) for refractory disease, one (16.6%) for relapsed disease after the suspension of methotrexate treatment dueto liver toxicity, and one (16.6%) due to dyspesia. A complete hematologic response was achieved in all cases, and a complete molecular response was achieved in five out six cases (83.3%). During a mean follow-up period of 12 months, both the progression-free survival and overall survival rates were 100%. CONCLUSION: T-cell large granular lymphocytic leukemia demonstrated a high rate of complete hematologic and molecular response to fludarabine, with excellent compliance and tolerability rates. To confirm our results in this rare disease, we believe that fludarabine should be tested in clinical trials as a first-line treatment for T-cell large granular lymphocytic leukemia.

Infection profile of patients undergoing autologous bone marrow transplantation in a Brazilian institution / Perfil de infecção em pacientes submetidos a transplante autólogo de medula óssea em uma instituição brasileira

CONTEXT AND OBJECTIVE: Hematopoietic stem cell transplantation (HSCT) has been widely used for treating oncological and hematological diseases. Although HSCT has helped to improve patient survival, the risk of developing infection during hospitalization is an important cause of morbidity and mortality. This study aimed to analyze the infection profile during hospitalization and the associated risk factors among patients undergoing autologous HSCT at the University Hospital, Universidade Federal de Juiz de Fora. DESIGN AND SETTING: This was a cross-sectional study on patients undergoing autologous HSCT at a public university hospital. METHODS: Patients with febrile neutropenia between 2004 and 2009 were retrospectively evaluated regarding their infection profile and associated risk factors. RESULTS: Infection occurred in 57.2 percent of 112 patients with febrile neutropenia. The main source of infection was the central venous catheter (25.9 percent). Infection was chiefly due to Gram-positive bacteria, although Gram-negative-related infections were more severe and caused a higher death rate. Sex, age, skin color, nutritional status and underlying disease were not associated with the development of infection. Patients with severe mucositis (Grades III and IV) had a higher infection rate (P < 0.001). Patients who developed pulmonary complications during hospitalization had higher infection rates (P = 0.002). Infection was the main cause of death (57.1 percent) in the study sample. CONCLUSION: Strategies aimed at reducing infection-related mortality rates among patients undergoing autologous HSCT are necessary.

CONTEXTO E OBJETIVO: O transplante de células-tronco hematopoiéticas (TCTH) vem sendo amplamente utilizado no tratamento das doenças onco-hematológicas. Embora o TCTH tenha colaborado para a melhora na sobrevida dos pacientes, o risco de desenvolver infecção no período de internação é uma importante causa de morbi-mortalidade. O presente estudo teve como objetivo analisar o perfil das infecções no período de internação e os fatores de risco associados entre os pacientes submetidos ao TCTH autólogo, no Hospital Universitário da Universidade Federal de Juiz de Fora. TIPO DE ESTUDO E LOCAL: Trata-se de um estudo transversal sobre pacientes submetidos a transplante autólogo, em um hospital público universitário. MÉTODOS: Foram analisados retrospectivamente os pacientes que apresentaram neutropenia febril no período de 2004 a 2009, com relação ao perfil infeccioso e os fatores de risco associados. RESULTADOS: A infecção foi determinada em 57,2 por cento dos 112 pacientes com neutropenia febril. A principal fonte de infecção foi o cateter venoso central (25,9 por cento). A infecção ocorreu principalmente devido a bactérias Gram-positivas, apesar de as infecções causadas por bactérias Gram-negativas terem sido mais graves e causado maior taxa de morte. Sexo, idade, cor da pele, estado nutricional e doença de base não estiveram associados com o desenvolvimento da infecção. Pacientes com mucosite grave (graus III e IV) apresentaram maior taxa de infecção (P < 0.001). Os pacientes que desenvolveram complicações pulmonares durante a internação apresentaram maiores taxas de infecção (P = 0,002). A infecção foi a principal causa do óbito (57,1 por cento) na amostra estudada. CONCLUSÃO: São necessárias estratégias voltadas para a redução da taxa de mortalidade relacionada com infecção entre pacientes submetidos ao TCTH autólogo.

Displasia fibrosa: relato de caso e revisão de literatura / Fibrous dysplasia: story of case and revision of literature

A displasia fibrosa (DF) do osso é uma desordem congênita, não hereditária, do esqueleto e de caráter benigno, que cursa com amplo espectro de apresentação, variando do assintomático à dor óssea, fraturas de repetição, deformidades ósseas (fêmur em cajado de pastor e fácies leonina) e compressão de nervos cranianos. É comumente referida como uma doença óssea de alto turnover. Todos os casos contêm a mutação GNAS1. A DF apresenta duas formas: a monostótica, mais comum, e a poliostótica, mais rara, que quando acompanhada de manchas café-com-leite e puberdade precoce constitui a síndrome de McCune -Albright. O tratamento pode ser feito com medicamentos como bifosfonato ou de forma cirúrgica, objetivando-se a correção das lesões. Este trabalho relata o caso de um menino de cinco anos de idade cujos sinais e sintomas conduziam ao diagnóstico de DF. Além disso, faz revisão de literatura sobre uma doença pouco comum, com variada gama de diagnósticos diferenciais.

Background and Objectives: Fibrous Dysplasia (FD) of bone is a benign nothereditary congenital disorder of medullary bone maintenace in which bone undergoingphysiologic lysis is replaced by abnormal proliferation of fibrous tissue,resulting in assymmetric distortion and expantion of bone. It may be confined toa single bone (monostotic) or involve several bones (polyostotic). Prompt recognitionof this disease is important once it takes part in a wide group of differentialdiagnosis. This review is intended to provide clinicians with an understanding ofthe pathophysiology that underlies FD and its presentation forms. Methods: Thisarticle reviews and analyzes literature relevant to the pathophysiology and managementof FD and presents a case-study of a five-year-old boy who came downwith it. Methods include search of MEDLINE, and bibliographic search of currenttextbooks and journal articles. Results and Conclusions: The patient who wasinvestigated had, at the beginning, bone pain and other symptoms leading toa possible diagnosis of FD, which was confirmed by bone biopsy. He remainsasymptomatic.

Linfoma de Hodgkin e gestação: relato de caso e revisão de literatura / Hodgkin?s lymphoma and pregnancy: case report and literature review

JUSTIFICATIVA E OBJETIVOS: Os linfomas representam a quarta doença maligna mais frequente na gestação, sendo o linfoma de Hodgkin (LH) o tipo mais comum. O tratamento em gestantes é individualizado com radioterapia ou quimioterapia. A decisão de realizar o tratamento quimioterápico durante a gravidez deve ser avaliada à luz dos efeitos sobre a vida materna, caso o tratamento seja adiado. Evidências sugerem que a quimioterapia durante o primeiro trimestre de gravidez aumenta o risco de abortos espontâneos, morte fetal e malformações, sendo menor quando o tratamento é com monoquimioterapia. Ausência de estudos aleatórios e escassez da literatura fazem com que não existam evidências sólidas sobre qual a melhor conduta a ser tomada. O objetivo deste relato foi apresentar a evolução de uma paciente com diagnóstico de linfoma de Hodgkin. RELATO DO CASO: Paciente de 18 anos, 22 semanas de gestação, apresenta linfonodomegalia em fossa supraclavicular direita. A biópsia revela LH esclerose nodular, estádio clínico IIA. Tratada com vinblastina obtendo redução parcial da massa nos primeiros três ciclos, quando se estabilizou. Após o nascimento de criança a termo, iniciou-se o tratamento com driamicina, bleomicina, vinblastina e dacarbazina (ABVD). Após cinco ciclos, a tomografia de tórax revelou linfonodomegalias no mediastino e na fossa supraclavicular direita. Realizou-se radioterapia com Mini-Mantle, apresentando resposta local. Permanece em acompanhamento ambulatorial há dois meses, sem evidências da doença. CONCLUSÃO: Doenças linfoproliferativas como o LH não são frequentes durante a gestação, e poucas séries de casos discutem como abordar essas pacientes. Apesar de a apresentação clínica ser similar à das não grávidas, deve-se levar em consideração as interações medicamentosas com o período gestacional, os efeitos do tratamento no feto em desenvolvimento e no recém-nascido, além dos riscos e benefícios do tratamento materno.

BACKGROUND AND OBJECTIVES: Lymphomas are the fourth most frequent malignant illnesses diagnosed during pregnancy and Hodgkin's lymphoma (LH) is the most common one. Treatment during pregnancy is based on radio and chemotherapy. The decision of using chemotherapy during pregnancy should be weighed against the effect of the treatment delay on maternal survival. The existing data shows that chemotherapy during the first trimester increases the risk of fetal or neonatal death and malformed infants. In these cases the lack of randomized studies as well as lack literature evidence difficults the choice of the best treatment procedure. This case report objective is to describe the evolution of a patient diagnosed with Hodgkin's lymphoma. CASE REPORT: 18 year-old woman, presented lymphonodemegaly in the right supraclavicular fossa by the 22nd week of pregnancy. Biopsy showed HL nodular sclerosis at stage IIA. After treatment with vinblastine the patient showed partial reduction of the tumor mass in the first 3 cycles, after which stabilization was observed. After successful delivery, adriamycin, bleomycin, vinblastine, dacarbazina (ABVD) was initiated and after 5 cycles, thorax tomography was performed and evidenced lymphonodemegaly in the mediastinum and in the right supraclavicular fossa. The patient underwent Mini-Manthle radiotherapy presenting local regression. She has been evaluated for 2 months without evidence of recurrence. CONCLUSION: Lymphoprolipherative disorders like HD aren't frequent during pregnancy, and only a few case series discuss the approach of these patients. Despite the clinical presentations being similar to those in non-pregnant patients, drug interaction during pregnancy, the effects of treatment on the developing fetus and on the newborn, and also the risks and benefits of the maternal treatment, should be taken in consideration.

Bcl-2 protein frequency in patients with high-risk diffuse large B-cell lymphoma / Frequência da proteína Bcl-2 em pacientes com linfoma difuso de grandes células B de alto risco

CONTEXT AND OBJECTIVE: Gene expression and immunohistochemical profiling of diffuse large B-cell lymphoma (DLBCL) have revealed important prognostic subgroups: germinal center B-cell-like (GCB-like) DLBCL and activated B cell-like (ABC-like) DLBCL. Although few reports on high-risk DLBCL are available, the prognosis for the GCB-like subgroup has been shown to be better than that of the ABC-like subgroup. The role of Bcl-2 as a predictor of survival in DLBCL cases is unclear and its expression varies between the two subgroups of DLBCL. In this study, we analyzed the frequency and prognostic impact of Bcl-2 protein expression in high-risk DLBCL cases. DESIGN AND SETTING: Retrospective cohort study among DLBCL patients treated at Hospital das Clínicas, Faculdade de Medicina da Universidade de São Paulo (HC-FMUSP). METHODS: The prognostic impact of the expression of the proteins CD10, Bcl-6, MUM1 (multiple myeloma oncogene-1) and Bcl-2 on high-risk DLBCL cases was evaluated by means of immunohistochemistry. Seventy-three patients aged 18-60 years were evaluated for all these markers. RESULTS: Twenty-four cases (32.9 percent) were GCB-like and 49 (67.1 percent) were ABC-like, with no difference regarding complete remission, disease-free survival or overall survival rates. Twenty-seven patients (37 percent) showed Bcl-2 expression, which was the only independent factor predicting a worse prognosis for overall survival according to multivariate analysis. CONCLUSION: Bcl-2 protein was expressed in 37 percent of the high-risk DLBCL patients, without any difference between the ABC-like DLBCL and GCB-like DLBCL cases.

CONTEXTO E OBJETIVO: A expressão gênica e imunoistoquímica do linfoma difuso de grandes células B (LDGCB) vem permitindo a identificação de importantes subgrupos prognósticos: LDGCB do centro germinativo (CG) e LDGCB de células B ativadas (CBA). Entretanto, existem poucos dados disponíveis com LDGCB de alto risco, sendo o prognóstico dos LDGCB do CG melhor que os LDGCB de CBA. A participação do Bcl-2 como preditor de sobrevida nos LDGCB não é clara e sua expressão é variável entre os dois subgrupos de LDGCB. Neste estudo é avaliada a frequência e o prognóstico da expressão da proteína Bcl-2 em LDGCB de alto risco. TIPO DE ESTUDO E LOCAL: Estudo de coorte retrospectivo realizado entre portadores de LDGCB tratados no Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo. MÉTODOS: Foi avaliado o impacto prognóstico da expressão das proteínas CD10, Bcl-6, MUM1 (multiple myeloma oncogene-1) e Bcl-2 por imunoistoquímica em LDGCB de alto risco. Foram avaliados, para todos os marcadores, 73 pacientes com idade de 18 a 60 anos. RESULTADOS: Vinte e quatro (32,9 por cento) pacientes foram classificados como LDGCB do CG e 49 (67,1 por cento) como LDGCB de CBA, sem diferença nas taxas de remissão completa, sobrevida livre de doença e sobrevida global. Vinte e sete (37 por cento) apresentaram expressão de Bcl-2, o qual foi o único fator preditivo independente de pior prognóstico de sobrevida global à análise multivariada. CONCLUSÃO: A expressão da proteína Bcl-2 ocorreu em 37 por cento dos portadores de LDGCB de alto risco, sem diferença entre os subgrupos de LDGCB do CG ou de CBA.

Risk stratification for indolent lymphomas / Estratificação de risco dos linfomas indolentes

Indolent B-cell lymphomas account for approximately 40 percent of all non-Hodgkin lymphomas (NHLs). Advances in technology have contributed to improvements in the diagnosis and classification of indolent non-Hodgkin lymphomas. Follicular Lymphomas are the most common although the frequency varies significantly throughout the world. The description of the Follicular Lymphoma International Prognostic Index (FLIPI) was an important step in identifying patient subgroups, but its use in the clinical practice has not been established yet. The use of a larger number of paraffin active monoclonal antibodies for immunohistochemistry, molecular cytogenetic studies including standard cytogenetics, multi-color fluorescence in-situ hybridization (FISH), polymerase chain reaction and locus-specific fluorescence insitu hybridization as well as developments in high-resolution techniquesincluding microarray gene expression profiling allow more accurate diagnosis andprecise definition of biomarkers of value in risk stratification. The identification ofdiseasespecific gene lists resulting from expression profiling provides a number ofpotential protein targets that can be validated using immunohistochemistry. Analysesof gene expression profiles or constitutive gene variations may also provide additional insight for prognostication in the near future. A comprehensive understanding of the biology of these distinct lymphoid tumors will allow us to identify novel diseaserelated genes and should facilitate the development of improved diagnosis, outcome prediction, and personalized approaches to treatment.

Os linfomas de células B indolentes representam aproximadamente 40 por cento do total de linfomas não Hodgkin (LNHs). O avanço das tecnologias novas tem contribuído para a melhora no diagnóstico e classificação dos LNH indolentes. O linfoma folicular é o mais comum e sua frequência varia significantemente pelo mundo. Adescrição do Índice Internacional de Prognóstico dos linfomas folicular (FLIPI) representa um passo importante na identificação de subgrupos de pacientes, mas seu uso na prática clínica ainda necessita ser estabelecido. O uso de um número maior de anticorpos monoclonais para imunoistoquímica, estudo citogenético incluindo citogenética convencional ou hibridização in-situ por fluorescência (FISH), bem como o desenvolvimento de técnicas de alta resolução incluindo a expressão por microarray possibilita maior acurácia no diagnóstico e definição precisa dos biomarcadores com valor na estratificação de risco. A identificação de genes específicos para os diversos tipos de linfomas permite o reconhecimento de potenciais proteínas alvo que podem ser validadas usando imunoistoquímica. Análises da expressão do perfil de genes ou variações genéticas constitutivas pode também prover conhecimentos adicionais para o prognóstico em um futuro próximo. Um entendimento da biologia desses distintos tumores linfoides permite-nos identificar novos grupos de genes relacionados à doença e deve facilitar o desenvolvimento diagnóstico, predizendo a evolução e permitindo tratamentos personalizados.

Amiloidose sistêmica primária AL: relato de caso e considerações associadas / Primary systemic AL amyloidosis: case report and associated considerations

Amiloidose não é uma única doença, e sim, uma condição presente em um grupo de doenças que tem em comum a deposição extracelular patológica de proteínas insolúveis em órgãos ou tecidos. Todas as fibrilas amiloides compartilham uma mesma estrutura secundária, a conformação em folha -pregueada, e um componente não fibrilar idêntico, a pentraxina amiloide sérica P (APS). Relatamos o caso de um paciente com 62 anos, portador de amiloidose sistêmica do tipo AL. O diagnóstico foi constatado através de exames histopatológicos e de imagens. Após diagnóstico, paciente foi submetido a seis ciclos de quimioterapia com vincristina, adriamicina e dexametasona (VAD). Após o qual, foi realizado manutenção com ciclofosfamida, 600mg por um dia, e dexametasona, 40mg por quatro dias repetidos a cada 28 dias. Paciente evoluiu com melhora da sintomatologia e retorno às suas atividades habituais.

Amyloidosis is a condition inherent to a group of diseases, which exhibit the common feature of pathological extracellular deposition of insoluble proteins in organs or tissues. All amyloid fibrils share the same secondary structure, the ?-pleated sheet conformation, and a nonfibrillar identical component, the serum amyloid pentraxin (SAP). We report a 62-year-old man with systemic AL amyloidosis. Diagnosis was made through histopathology and imaging. The patient underwent 6 cycles of vincristine, adriamycin and dexamethasone (VAD), with maintenance with cyclophosphamide 600mg for 1 day and dexamethasone 40mg/day for 4 days every 28 days. The patient was relieved of his symptoms and returned to his daily activities.

Estudo observacional do prognóstico e terapêutica dos portadores de linfoma difuso de grandes células B de IPIa de risco intermediário alto e alto / Observational Study of prognosis and therapeutics of Diffuse Large B Cell Lymphoma patients with high intermediate to high aIPI risk

Elaboramos projeto de pesquisa para verificar o papel dos marcadores CD10, Bcl-6, MUM-1, Bcl-2 e p63 e do transplante de medula óssea autólogo (ATMO), em primeira remissão completa, em portadores de linfoma difuso de grande célula B de risco intermediário alto e alto. A proteína Bcl-2 foi positiva em 27 (37%) pacientes e foi o único fator preditivo independente para sobrevida global (SG) à análise multivariada. As SG e sobrevida livre de doença (SLD) para os 16 pacientes que receberam ATMO foram de 75% e 85,2 por cento, respectivamente, com diferença estatisticamente significativa para SLD (p = 0,015). Concluímos que o ATMO foi seguro e capaz de melhorar a sobrevida, e que a expressão de Bcl-2 pode ser utilizada na programação terapêutica inicial destes pacientes...

A research project was designed in order to analyse the impact of the expression of CD10, Bcl-6, MUM-1, Bcl-2 and p63 markers and the role of autologous stem cell transplantation (ASCT) in first complete remission for diffuse large B cell lymphoma with high intermediate to high risk. Bcl-2 expression was found in 37% (27) of the patients and was the single independent predicting factor of overall survival (OS) prognosis according to multivariate analysis. OS and disease free survival (DFS) were of 75% and 85.2% respectively for the group of 16 patients treated with ASCT, with statistically significant difference for DFS (p=0.015). In this study ASCT was found to be a safe procedure for improving survival rates and the expression of Bcl-2 protein was found to be useful as one of the variables to be analysed in the therapeutic approach to these patients...

Primary biliary cirrhosis and myopathy: an uncommon association

Primary biliary cirrhosis (PBC) is a cholestatic liver disease, which is characterized by a chronic inflammatory destruction of intrahepatic bile ducts. It is a rare disorder whose precise etiology is still to be elucidated. Even though the liver is the principal target of PBC, other organ systems also might be affected. Muscular involvement has rarely been described in this disease, and in the majority of cases, muscular weakness has been interpreted as polymyositis. We report the case of a 48-year-old woman suffering from classic PBC, in association with a myopathy whose histological features are distinct from the cases reported before. We also performed a MEDLINE research for PBC and concomitant muscular diseases