Burden of multiple myeloma in China: an analysis of the Global Burden of Disease, Injuries, and Risk Factors Study 2019 / 中华医学杂志(英文版)

BACKGROUND@#There is limited data to comprehensively evaluate the epidemiological characteristics of multiple myeloma (MM) in China; therefore, this study determined the characteristics of the disease burden of MM at national and provincial levels in China.@*METHODS@#The burden of MM, including incidence, mortality, prevalence, and disability-adjusted life years (DALYs), with a 95% uncertainty interval (UI), was determined in China following the general analytical strategy used in the Global Burden of Disease, Injuries, and Risk Factors Study 2019. The trends in the burden of MM from 1990 to 2019 were also evaluated.@*RESULTS@#There were an estimated 347.45 thousand DALYs with an age-standardized DALY rate of 17.05 (95% UI, 12.31-20.77) per 100,000 in 2019. The estimated number of incident case and deaths of MM were 18,793 and 13,421, with age-standardized incidence and mortality rates of 0.93 (95% UI, 0.67-1.15) and 0.67 (95% UI, 0.50-0.82) per 100,000, respectively. The age-specific DALY rates per 100,000 increased to more than 10.00 in the 40 to 44 years age group reaching a peak (93.82) in the 70 to 74 years age group. Males had a higher burden than females, with approximately 1.5- to 2.0-fold sex difference in age-specific DALY rates in all age groups. From 1990 to 2019, the DALYs of MM increased 134%, from 148,479 in 1990 to 347,453 in 2019.@*CONCLUSION@#The burden of MM has doubled over the last three decades, which highlights the need to establish effective disease prevention and control strategies at both the national and provincial levels.

Trabalho rural associado a cânceres linfohematopoiéticos em hospital público de referência: estudo caso-controle, Ceará, Brasil, 2019-2021 / Rural labor associated with lymphohematopoietic cancers in a public reference hospital: a case-control study, Ceará, Brazil, 2019-2021 / Trabajo rural asociado a cánceres linfohematopoyéticos en un hospital público de referencia: estudio de caso control, Ceará, Brasil, 2019-2021

Trata-se de um estudo do tipo caso-controle pareado com o objetivo de verificar a associação entre ocupações e cânceres linfohematopoiéticos em um hospital público de referência no Estado do Ceará, Brasil, durante 2019-2021. O grupo caso foi constituído por pacientes hematológicos que apresentavam mieloma múltiplo, leucemias ou linfomas não Hodgkin, acompanhados pelo serviço social de uma unidade hospitalar de transplante de medula óssea (n = 114), enquanto indivíduos comparáveis de unidade hospitalar distinta constituíram o grupo controle (n = 114), formando pares 1:1. O diagnóstico foi efetuado por equipe médica e as variáveis foram aferidas por acesso aos registros hospitalares. Comparamos os grupos em regressão logística condicional bivariada e ajustada por região de residência. Entre os resultados, destacamos que o câncer de maior prevalência no grupo caso foi o mieloma múltiplo (43,9%), seguido pelas leucemias (43%) e por linfomas não Hodgkin (13,2%). Proporções de ocupações, zona de residência, abastecimento de água e Superintendência Regional de Saúde de residência apresentaram diferença estatisticamente significante entre os grupos caso e controle. Verificamos que trabalhadores rurais possuíam maiores chances de apresentar os cânceres estudados (ORbruto = 5,00, IC95%: 1,91; 13,06 e ORajustado = 3,38, IC95%: 1,20; 9,54), enquanto trabalhadores do comércio apresentaram menores chances (ORbruto = 0,26, IC95%: 0,10; 0,70 e ORajustado = 0,30, IC95%: 0,10; 0,88). Os achados deste estudo possibilitam reflexões sobre o processo de adoecimento dos trabalhadores rurais e refletem o potencial dos serviços de saúde em contribuir com investigações sobre exposições ocupacionais.

This is a paired case-control study that sought to verify the association between professions and lymphohematopoietic cancers in a public reference hospital in the State of Ceará, Brazil, during 2019-2021. Case group consisted of hematological patients with multiple myeloma, leukemias or non-Hodgkin lymphomas, monitored by the social service of a bone marrow transplant hospital unit (n = 114), whereas comparable individuals from a different hospital unit made up the control group (n = 114), forming 1:1 pairs. Diagnosis was performed by a medical team and the variables were measured by accessing hospital records. Group comparison was conducted using bivariate conditional logistic regression and adjusted by region of residence. Results show that multiple myeloma (43.9%), leukemias (43%) and non-Hodgkin lymphomas (13.2%) were the cancer with the highest prevalence in the case group. Proportions of jobs, area of residence, water supply and Regional Superintendence of Health showed statistically significant difference between the case and control groups. Rural workers were more likely to present the cancers studied (ORraw = 5.00, 95%CI: 1.91; 13.06 and ORadjusted = 3.38, 95%CI: 1.20; 9.54), whereas trade workers had lower odds (ORraw = 0.26, 95%CI: 0.10; 0.70 and ORadjusted = 0.30, 95%CI: 0.10; 0.88). The findings allow us to reflect on the process of illness among rural workers and illustrate the potential of health services to contribute to investigations on occupational exposures.

Se trata de un estudio del tipo caso control apareado con el objetivo de verificar la asociación entre ocupaciones y cánceres linfohematopoyéticos en un hospital público de referencia en el estado de Ceará, Brasil, durante el período 2019-2021. El grupo caso estaba formado por pacientes hematológicos que presentaban mieloma múltiple, leucemias o linfomas no Hodgkin, bajo seguimiento por parte del servicio social de una unidad hospitalaria de trasplante de médula ósea (n = 114), mientras que individuos comparables de otra unidad hospitalaria constituyeron el grupo control (n = 114), formando pares 1:1. El diagnóstico fue realizado por un equipo médico, y las variables se midieron accediendo a los registros hospitalarios. Comparamos los grupos en regresión logística condicional bivariada y ajustada por región de residencia. Entre los resultados, destacamos que el cáncer de mayor prevalencia en el grupo caso fue el mieloma múltiple (43,9%), seguido de las leucemias (43%) y los linfomas no Hodgkin (13,2%). Las proporciones de ocupaciones, zona de residencia, abastecimiento de agua y Superintendencia Regional de Salud de residencia presentaron diferencia estadísticamente significativa entre los grupos caso y control. Constatamos que los trabajadores rurales tenían más probabilidades de padecer los cánceres estudiados (ORcrudo = 5,00, IC95%: 1,91; 13,06 y ORajustado = 3,38, IC95%: 1,20; 9,54), mientras que los trabajadores del comercio presentaron menos posibilidades (ORcrudo = 0,26, IC95%: 0,10; 0,70 y ORajustado = 0,30, IC95%: 0,10; 0,88). Los hallazgos de este estudio permiten reflexiones sobre el proceso de enfermedad de los trabajadores rurales y reflejan el potencial de los servicios de salud para contribuir a las investigaciones sobre exposiciones ocupacionales.

Perfil epidemiológico de pacientes diagnosticados com mieloma múltiplo em hospital de referência paraneoplasias malignas hematológicas / Epidemiological profile of patients diagnosed with multiple myeloma at a reference hospital for haematological malignancies

Objetivo: Analisar dados epidemiológicos, clínicos e laboratoriais de pacientes diagnosticados com mieloma múltiplo (MM) atendidos no Hospital Regional de Mato Grosso do Sul. Métodos: Estudo retrospectivo do tipo descritivo observacional, com seleção de dados clínicos e laboratoriais, envolvendo pacientes atendidos de 2013 a 2017. Resultados: Foram incluídos no estudo 44 pacientes. A mediana de idade foi de 63 anos, predominando o sexo masculino. Hipertensão arterial sistêmica e dor lombar foram, respectivamente, a comorbidade e manifestação clínica mais comum ao diagnóstico. Grande parcela dos pacientes possuía lesões líticas. As principais alterações laboratoriais foram anemia, elevação de ureia, creatinina e hiperproteinemia. Mais da metade dos pacientes evoluiu para óbito, sendo choque séptico e parada cardiorrespiratória as principais causas. A média de sobrevida dos pacientes foi de 25,81 meses. Correlações significativas foram estabelecidas entre os fatores idade, infecções, fraqueza, hiporexia, insuficiência renal, elevação de ureia, creatinina, proteínas totais, globulinas e hipoalbuminemia ao diagnóstico e um maior risco de óbito em menos de um ano. Conclusão: Os resultados obtidos irão contribuir para o maior conhecimento dessa neoplasia na população sul-matogrossense, impactando diretamente no diagnóstico precoce e manejo da doença.

Objective: To analyze epidemiological, clinical and laboratory data of patients with multiple myeloma (MM) who attended the Regional Hospital of Mato Grosso do Sul. Methods: We conducted a retrospective study of descriptive observational design, by collecting clinical and laboratory data, involving patients hospitalized from 2013 to 2017. Results: Forty- four patients were included in the study. The median age was 63 years, with a predominance of males. Systemic arterial hypertension and low back pain were, respectively, the most common comorbidity and clinical manifestation. A large portion of patients had lytic bone lesions. The main laboratory findings were anemia, increase of urea, creatinine and hyperproteinemia. More than half of patients died, being septic shock and cardiorespiratory arrest the main causes of death. The mean patient survival rate was 25.81 months. Significant correlations were established between the factors age, infections, weakness, hyporexia, renal insufficiency, increase of urea, creatinine, total proteins, globulins and hypoalbuminemia at diagnosis and a higher risk of death in less than one year. Conclusion: The obtained results will contribute to a better knowledge about this neoplasm in the population of Mato Grosso do Sul, impacting directly on the early diagnosis and management of this disease.

Importancia de las alteraciones genéticas del mieloma múltiple / Importance of the genetic alterations of multiple myeloma

Introducción: El mieloma múltiple (MM) es una enfermedad que va precedida por una fase previa conocida como gammapatía monoclonal de significado incierto (GMSI); en esta última existen varias anormalidades citogenéticas, que permiten la progresión a MM, entre estas encontramos reordenamientos primarios del gen de la cadena pesada de la inmunoglobina (IGH), además de células hiperdiploides. Desarrollo: Las alteraciones cromosómicas en el MM se pueden clasificar en dos grupos principales: las que involucran las translocaciones del locus IGH ubicado en el cromosoma 14q32 y cuyos principales reordenamientos se dan entre las regiones cromosómicas 11q13, 16q23, 4p16.3, 6p21 y, un segundo grupo caracterizado por los desequilibrios genómicos. Los pacientes con translocaciones de la IGH, muestran un pronóstico diferente en dependencia del tipo de reordenamiento cromosómico. La t(4;14)(p16;q32) y t(14;16)(q32;q23) se asocian a un mal pronóstico, mientras que los pacientes con t(11;14) (q13;q32) tiene un buen pronóstico de la enfermedad en ausencia de otras anormalidades genéticas. En el grupo con desequilibrio genómico se encuentran deleciones, amplificaciones, y células con números anormales de cromosomas (hiperdiploidas y no hiperdiploides); casi siempre asociadas a mal pronóstico ya que muchas de estas alteraciones involucran perdida de material genómico relacionado con el control de ciclo celular y progresión de la enfermedad, como son las deleciones de los cromosomas 1,13 y 17. Los pacientes con trisomías de los cromosomas impares 1, 3, 5, 7, 9, 11, 15, 19,21 suelen tener un mejor pronóstico y una tasa mayor de sobrevivencia(AU)

Introduction: Multiple myeloma (MM) is a disease that is preceded by a previous phase known as monoclonal gammopathy of uncertain significance (MGUS); in this latter there are several cytogenetic abnormalities, which allow the progression to MM, among these we find primary rearrangements of the heavy chain gene of the immunoglobin (IGH), in addition to hyperdiploid cells. Development: Chromosomal alterations in MM can be classified into two main groups, those involving the translocations of the IGH locus located on chromosome 14q32 and whose main rearrangements occur between the chromosomal regions 11q13, 16q23, 4p16.3, 6p21, and a second group which is characterized by genomic imbalances. Patients with translocations of the IGH, show a different prognosis depending on the type of chromosomal rearrangement, the t(4; 14)(p16; q32) and t(14; 16)(q32; q23) are associated with a poor prognosis while patients with t(11; 14)(q13; q32) have a good prognosis of the disease in the absence of other genetic abnormalities. Within the genomic imbalances we find deletions, amplifications, and cells with abnormal numbers of chromosomes (hyperdiploids and not hyperdiploid), these almost always associated with poor prognosis since many of these alterations involve loss of genomic material related to cell cycle control and progression of the disease, such as deletions of chromosomes 1,13 and 17. Patients with trisomies of odd chromosomes 1, 3, 5, 7, 9, 11, 15, 19,21 usually have a better prognosis and a higher survival rate(AU)

Perfil de pacientes com diagnóstico patológico de mieloma múltiplo em hospital de ensino / Profile of patients with multiple myeloma pathological diagnosis in a tea-ching hospital

Introdução: Plasmocitoma é um tumor maligno, originado da proliferação irreversível e autônoma dos plasmócitos, podendo se apresentar como massa circunscrita ou infiltração difusa. Quando há mais de um tumor de células plasmáticas, ocorre o chamado Mieloma Múltiplo. Essa neoplasia maligna de células B apresenta produção exagerada de uma das imunoglobulinas de cadeias leves Kappa ou Lambda. Objetivo: Avaliar possíveis associações entre dados epidemiológicos e a carga tumoral em pacientes com Mieloma Múltiplo. Material eMétodos: Trata-se de um estudo transversal, quantitativo, descritivo, realizado em prontuários de pacientes diagnosticados com Mieloma Múltiplo no Laboratório de Patologia do Hospital de Base do município de São José do Rio Preto, São Paulo, entre 2010 e 2014. Foram consideradas as variáveis: sexo, idade, presença de cadeia leve (Kappa, Lambda, ambas e não especificada), carga tumoral (leve, moderada e acentuada). Resultados:Analisamos 42 laudos de pacientes diagnosticados com Mieloma Múltiplo, sendo que 69% dos pacientes apresentaram idade igual ou superior a 60 anos, presença de imunoglobulina de cadeia leve Kappa em 59% e carga tumoral acentuada em 67% dos casos. Em relação ao sexo, observamos que o nível de carga tumoral acentuada ocorreu de maneira similar entre homens e mulheres. As mulheres diagnosticadas com carga tumoral leve ou moderada configuraram 71% dos pacientes, do estudo. Conclusão: A idade e sexo não constituem fator de risco para o Mieloma Múltiplo na amostragem avaliada, este fato pode estar relacionado ao tamanho amostral.

Introduction: Plasmocytoma is a malignant tumor, originated from the irreversible and autonomous proliferation of the plasmacytes, being able to present as circumscribed mass or diffuse infiltration. When there is more than one plasma cell tumor, the so-called Multiple Myelomas occurs. This malignant B cell neoplasm exhibits overproduction of one of the Kappa or Lambda light chain. Objective: To evaluate possible ssociation of epidemiological data with tumor burden in patients with multiple myelomas. Patients and Methods:It is a cross-sectional study conducted with medical records of patients diagnosed with multiple myeloma by the Pathology Laboratory in Hospital de Base in the city of São José do Rio Preto, São Paulo, between 2010 and 2014. We considered the variables gender, age, light chain presence (Kappa, Lambda, both and unspecified), and tumor burden (mild, moderate or severe). Results: We analyzed 42 medical records of patients diagnosed with multiple myelomas. Of these, 69% of patients were aged 60 and over. Immunoglobulin Kappa light chain was found in 59% patients, and severe tumor burden in 67% of them. Regarding sex, we observed that severe tumor burden level occurred in a similar manner in men and women, without significant associations. Women diagnosed with mild to moderate tumor burden corresponded to 71% of patients, but no significant association was found. Conclusion: Age and sex were not a risk factor for multiple myelomas in this evaluated sample. This may be due to the sample size evaluated.

Comportamiento de las inmunoglobulinas en el seguimiento de pacientes con mieloma múltiple. Hospital Universitario Comandante Faustino Pérez. Matanzas / Behavior of the immunoglobulines in the follow-up of patients with multiple mieloma. University Hospital Comandante Faustino Pérez. Matanzas

Introducción: las gammapatías monoclonales son un grupo de desórdenes caracterizados por la proliferación de un clon de células plasmáticas que sintetiza una inmunoglobulina monoclonal, dentro de las que se encuentra como ejemplo de neoplasia de células plasmáticas el mieloma múltiple, entidad que se caracteriza por la producción anormal de inmunoglobulinas. Objetivo: describir el comportamiento de las inmunoglobulinas en el seguimiento de pacientes con mieloma múltiple, en el Servicio de Hematología del Hospital "Comandante Faustino Pérez" de Matanzas. Materiales y Métodos: estudio descriptivo-prospectivo-longitudinal. El universo de estudio fue abierto y la muestra fueron todos los pacientes con mieloma múltiple de debut, durante el período del 1ro de junio del 2013 al 31 de mayo del 2014, a los cuales se les realizó la determinación de inmunoglobulinas IgA, IgG e IgM. Resultados: los valores de IgA e IgG disminuidos presentaron una respuesta al tratamiento muy buena ya desde los seis meses, aunque continuó mejorando a los 12 meses. La IgM a los seis meses de tratamiento se modificó hacia valores aumentados clasificando como refractario revirtiéndose este comportamiento a los 12 meses alcanzando un 13% más que antes del tratamiento. Conclusiones: los marcadores de mejor respuesta al tratamiento fueron las inmunoglobulinas IgA e IgG (AU).

Introduction: monoclonal gammopathies are a group of disorders characterized for the proliferation of a clone of plasmatic cells that synthesize a monoclonal immunoglobulin; among them, an example of plasmatic cells neoplasias is the multiple myeloma, an entity characterized by the abnormal production of immunoglobulins. Objective: to describe the behavior of immunoglobulins in the follow-up of patients with multiple myeloma in the Hematology Service of the University Hospital "Comandante Faustino Pérez", of Matanzas. Materials and methods: longitudinal-prospective-descriptive study. The universe of study was open and the sample was all the patients with debuting multiple myeloma, during the period from June 1st 2013 to May 31st 2014, to whom the determination of IgA, IgG and IgM immunoglobulins was carried out. Results: the diminished values of IgA and IgG showed a very good answer to the treatment after six and 12 months. At the sixth months of the treatment the IgM was modified to augmented values classifying as refractory; this behavior relapsed at the twelfth month, reaching 13 % more than before the treatment. Conclusions: the markers of better answer to the treatment were the IgA and IgG immunoglobulins (AU).

Caracterización clínica y supervivencia de pacientes con múltiple mieloma en un estado del noreste Brasil / Clinical characterization and survival of patients with multiple myeloma in a state of northeast brazilian

Introduction: The annual prevalence of Multiple Myeloma in patients aged between 65 and 74 years old is 31 cases for every 100 000 people and increases to 46 cases per 100 000 people in patients older than 75 years old. Objectives : To clinically characterize patients with multiple myeloma treated at a referral center in the state of Rio Grande do Norte, Brazil, and to estimate their survival. Methods : Retrospective cohort study. For data collection, secondary sources listed in the cadastral database and complementation with data records was used. The descriptive analysis was performed by using the Epi Info program, version 3.5.2 and the survival analysis used Statistic Package for Social Sciences (SPSS) software version 22. Results : Out of the 39 patients studied, 17 (43.6 percent) were male, and 22 (56.4 percent) were female, with an average age of 66.3 years old. The main medication therapeutic regimen was the combination of Cyclophosphamide, Thalidomide, and Dexamethasone (CTD), as the most medical prescription used in 63.6 percent of cases. Only 38.5 percent had other comorbidities and 46.2 percent of patients developed plasmacytoma. In the International Staging System (ISS), stage III prevailed with 30.8 percent among the evaluated patients. The cumulative probability of global survival rate showed a disease with a survival rate of 50 percent of the sample in about 36 months (three years) after diagnosis is confirmed(AU)

Introducción: la prevalencia anual de mieloma múltiple en pacientes entre 65 y 74 años de edad es de 31 casos por cada 100 000 personas y aumenta a 46 casos por 100 000 personas en pacientes mayores de 75 años. Objetivos: caracterizar clínicamente a los pacientes con mieloma múltiple tratados en un centro de referencia en el estado de Rio Grande do Norte, Brasil, y estimar su supervivencia. Métodos: estudio de cohorte, retrospectivo. Para la recopilación de datos, se utilizaron fuentes secundarias de la base de datos catastral y complementación con registros de datos. El análisis descriptivo se realizó mediante el programa Epi Info, versión 3.5.2 y el análisis de supervivencia del software estadístico SPSS 22. Resultados: de los 39 pacientes estudiados, 17 (43,6 por ciento) eran varones y 22 (56,4 por ciento), mujeres; con una edad promedio de 66,3 años. El principal régimen terapéutico de medicación fue la combinación de ciclofosfamida, talidomida y dexametasona (CTD), como la prescripción médica más utilizada en el 63,6 por ciento de los casos. Solo el 38,5 por ciento tenía otras comorbilidades y el 46,2 por ciento de los pacientes desarrolló plasmocitoma. En el International Staging System (ISS), prevaleció la etapa III (30,8 por ciento de los pacientes evaluados). La probabilidad acumulada de supervivencia global mostró una enfermedad con una tasa de supervivencia del 50 por ciento de la muestra en aproximadamente 36 meses (tres años) después de que se confirmó el diagnóstico(AU)

Myélome multiple avant 50 ans : 22 cas Brazzavillois

Le myélome multiple est classiquement une pathologie du sujet âgé. Nous rapportons dans une étude rétrospective; 22 patients atteints de proliférations plasmocytaires malignes (PPM) âgés de moins de 50. L'âge moyen de ces patients est de 39;2 ans (extrêmes 28 et 49). Parmi ces patients; on dénombre 4 âges de moins de 35 ans. Il s'agit de 13 femmes et 9 hommes. Les circonstances diagnostiques font démontrer que 17 patients sont admis au stade avance de la maladie (IIA et B) selon la starification internationale de Durie et Salmon. Les complications inaugurales telles que les atteintes neurologiques; l'insuffisance rénale aigue; les fractures pathologiques multiples; le syndrome anémique témoignent de l'agressivité de la symptomatologie initiale. L'infection évolutive a VIH/SIDA et l'infection latente a EBV sont associées a l'hémopathie maligne respectivement dans 2 cas chacune. Le plasmocyte solitaire a localisation maxillo-faciale est observé dans 1 cas. On note selon le type d'Immuniglobuline; 10 cas de myélome a IgG ; 7 cas q IgA et 4 cas de myélome a Bences-Jones. Sous poly chimiothérapie; 17 patients sur 22 ont survécu au-delà de 24 mois. Cette étude confirme la fréquence des PPM au sein de la race noire et rapporte certaines particularités de ces hémopathies malignes en Afrique noire qui se distinguent par l'originalité des formes classiques

Mieloma múltiplo no Brasil: aspectos clínicos, demográficos e validação do Sistema de Estadiamento Internacional (ISS) em pacientes brasileiros / Multiple myeloma in Brazil: clinicl and demographic aspects and International Staging System (ISS) validations for Brazilian patients

Durante o VI Simpóso Ítalo Brasileiro em Onco-Hematologia e Transplante de Medula Óssea apresentarei os resultados do estudo epidemiológico do mieloma múltiplo no Brasil. O mieloma múltiplo é a segunda neoplasia hemaológica mais frequente. Há poucos registros sobre a incidência e aspectos clínicos do mieloma múltiplo em grupos étnicos da Aérica Latina. No Brasil, por exemplo, a incidência de mielma múltiplo é desconhecida, uma vez que a doença não aparece nas estimtivas anuais fornecidas pelo Instituto Nacional de Câncer. A sobrevida mediana atual de pacientes com mieloma múltiplo é de aproximdamente três ano, mas há uma alta variabildade no prognóstico devido à heterogeneidade na biologia do mieloma múltiplo e nos fatores relacionados ao hospedeiro. O sistema de estadiamnto clínico de Durie-Salmon é preditivo de sobrevida global, permanecendo como o sistema de estadiamento mais amplamente utilizado por mais de 25 anos. Durante esse tempo, poucos parâmetros prognósticos novos surgiram com a beta2 microglobulina emergindo como o fator prognóstico mais poderoso, sendo simples e confiável na previsão de sobrevida. Estudos subsequentes sugeriram que a abumina era um outro indicaor prognóstico poderoso e de fácil aplicacação para o mieloma múltiplo. Recentemente, um painel internacional de investigadores apresentou o Sistema de Estadiamnto Internacional (International Staging System, ISS) par o mieloma múltiplo, uma escala de prognóstico que leva em conta a beta2 microglobulina sérica e a albumina como fatores significativos em análise multivariada, classificando então os pacientes em três grupos de risco. O Sistema ISS foi validado com pacientes da América do Norte, Europa e Ásia, mas não foi aplicado em um banco de dados do Brasil. Para avaliar os aspectos demográficos e clínicos dos pacientes com mieloma múltiplo no Brasil e observar se estas características são semelhantes às encontradas nos diferentes países da América do Sul e a outros países da Europa...

During the VI Italo-Brasilian Syposium on Onco-Hematology and Bone Marrow Transplatation, I will present the findings of the Epidemiological Study of Multiple Myeloma in Brazil. Multiple Myeloma is the second most frequent hematologic neoplasia. There are few records on the incidence and clinical aspects of multiple myeloma in ethic groups in Latin America. In Brasil, for example, the incidence of multiple myeloma is is unknown, as the disease does not appear in the annual estimates supplied by the National Cancer Institute. The current median survival of patients with multiple myeloma is approximately three years, but there is a high variability in the prognosis due to the heterogeneity of the biology of multiple myeloma and factors related to the host. The Durie-Salmon clinical staging system is predictive of the global survival, remaining as the staging sytem which has been most amply used for more than 25 years. During this fime, few new prognostic parameters have appeared, with beta-2-microglobulin emerging as the most powerful prognostic factor, it being a simple and realible forecast of survival . Subsequent studies suggested that albumin was another powerful indicator of easy application for multiple myeloma. Recently, an international panel of investigators presented the International Staging System, ISS, for multiple myeloma, a prognostic scale which takes serum beta-2-microglobulin and albumin into account as significant factors in multivariate analysis, thus classifying the patients into three risk groups. The ISS system was validated in patients in North America, Europe and Asia, but was not applied in a database in Brazil. To evaluated the demographic and clinical aspects of multiple myeloma patients in Brazil, and to observe if these characteristics are similar to the ones found in different countries of Latin America and other countries of Europe , North America and continents, we retrospectively analyzed all the cases of multiple...

Risk factors for multiple myeloma: evidence from the Japan Collaborative Cohort (JACC) study.

This study assessed the association of multiple myeloma (MM) with age, body mass index (BMI, kg/m(2)), physical activity, occupational history, and medical history for a Japanese cohort of 46,157 men and 63,541 women aged 40-79 years followed during 1988-2003 years. Cox proportional hazard model was mainly used to estimate the age and sex adjusted hazard ratio (HR) of MM including 95% confidence interval (CI) for both sexes. Same model, adjusted for age, was also used for each sex. In total, 98 MM deaths (men=49 and women=49) was observed for both sexes. Higher age groups (60-69 and 70-79 years) experienced significantly higher unadjusted HR of MM than the age group of 40-49 years. Men revealed significantly higher age-adjusted MM than women (HR=1.5; 95% CI=1.0-2.2). For both sexes, higher BMI of >or=30 kg/m(2)) (HR=2.8; 95% CI=1.0-7.7), walking <or=30 minutes/day (HR=2.0; 95% CI=1.2-3.4), worried about personal relationship in working place (HR=2.3; 95% CI=1.3-4.2), restricted own pace in working place (HR=1.9; 95% CI=1.0-3.4), and history of peptic ulcer (HR=1.7; 95% CI=1.0-2.7) significantly increased age and sex adjusted MM risk. Some of the above-mentioned significant associations became insignificant for age adjusted sex specific analyses. However, these findings should be validated by further epidemiologic studies in Japan before generalization.

Myeloma in young age.

Total 14 cases of myeloma in young age group (<40 years) have been reported out of 178 cases of myeloma in a time period of 7 years (1993-1999). Males predominated overfe males. Like adult myeloma, patients presented mostly with the backache, pain in pelvis, lower spine and weakness in about 60% of cases followed by swelling of bone in 40% of cases. One case presented with bleeding gum, malena and hepatosplenomegaly and was diagnosed as plasma cell leukemia. Radiological examination revealed lytic lesion in almost all the cases with fracture femur and rib in 28.57% of cases. Anaemia and raised ESR was noted in all the cases. Myeloma typing revealed IgG myeloma in 10 cases, light chain myeloma in 3 cases and IgA myeloma in one case. None of the patient was traceable after 2 years. Thus our study concludes that myeloma in the young age in India occurs in increased frequency and clinically presents just like adult and elderly myeloma, but serologically are predominantly of IgG type. There is also an increased frequency of solitary plasmacytoma as compared to adult myeloma.

Trasplante de células hematopoyéticas en mieloma múltiple / Hematopoietic stem cell transplantation in multiple myeloma

Multiple myeloma (MM) is the second most common hematologic malignancy, affecting approximately 14,000 new patients per year in the United States. For over four decades, the standard treatment for MM has been a regimen of melphalan combined with prednisone. Using this treatment modality, complete responses are rare, and 50% of patients have had disease that was resistant to chemotherapy. Attempts have been made to improve the outcome of MM by administering combinations of I. V. polichemotherapy, but these treatments are equivalent in terms of overall survival. High-dose therapy with peripheral blood stem cell support can be applied safely in these patients and achieves significantly higher complete remission rates as well as better event-free survival and overall survival. However, neither tumor-cell purging, positive selection, intensification of conditioning with additional chemotherapeutic agents, nor total body irradiation have been shown to improve outcome. The role of tandem transplantation with high-dose melphalan seems to be a good selection of treatment in hospitals having all resources. Future research will include the combination of the best remission-induction regimen with tandem transplants and maintenance treatments (thalidomide, idiotype or dendritic cell vaccination) that will sustain complete remission. Development of non-myeloablative allogeneic transplantation in order to exploit the graft-versus myeloma effect provides an alternative for patients who have a compatible donor. Combining all of these modalities with the new drugs developed few years ago (thalidomide, bortezomib, revlimid), we hope that MM will become a manageable chronic disease and perhaps a curable disease at least for 30% to 40% of the patients.

El mieloma múltiple (MM) es la segunda patología oncohematológica más frecuente. En Estados Unidos son diagnosticados anualmente 14,000 casos nuevos. En las últimas cuatro décadas el tratamiento estándar ha sido la combinación de melfalán y prednisona. Con este régimen raramente se logran remisiones completas y 50% de los pacientes no responden a esta terapia. Se han hecho intentos de mejorar los resultados combinando poliquimioterapia, pero la sobrevida global ha sido la misma. Al aplicar quimioterapia a dosis altas y rescate con trasplante de células hematopoyéticas se logra un mayor porcentaje de remisiones completas, asimismo, una mayor sobrevida libre de enfermedad y sobrevida global. La purga de células hematopoyéticas, selección positiva, intensificación del régimen de acondicionamiento con otras drogas o irradiación corporal total, no han demostrado utilidad en términos de sobrevida global. El doble trasplante autólogo de células hematopoyéticas parece ser una opción útil para hospitales que cuentan con la infraestructura y los recursos necesarios para realizarlo. En un futuro, la investigación deberá incluir el uso del mejor régimen de inducción a la remisión más doble trasplante autólogo y terapia de mantenimiento (talidomida o vacunas con células dendríticas), con la finalidad de al menos prolongar la remisión completa. El uso del trasplante alogénico no mieloablativo para provocar el efecto injerto contra mieloma parece una buena alternativa para los pacientes que tengan donador. Al combinar todas estas modalidades de tratamiento con las nuevas drogas desarrolladas en los últimos años (talidomida, bortezomid, revlimid), se espera que en un futuro el MM se convierta en una enfermedad crónica y curable en al menos 30 a 40% de los enfermos.

A case-control study of multiple myeloma in Japan: association with occupational factors.

Recently the incidence rate of multiple myeloma (MM) has increased in Japan. Epidemiologic efforts have suggested that certain occupational and chemical exposures are likely to increase the risk for MM. We therefore performed a case-control study of MM, examining occupational factors. Data for 57 cases and 57 controls were obtained from Sapporo Medical University Hospital and its affiliated hospitals in Hokkaido. Controls were matched to each case by gender, age (+/- 5 years) and hospital. Detailed information regarding several factors possibly related to MM was obtained by interviews in hospitals. Odds ratios (ORs) and 95% confidence intervals (95%CIs) were calculated with adjustment for cigarette smoking as confounding factor. The occupational category of agriculture and fishery showed a significant association with increased risk (OR = 5.89, 95% CI = 1.24-28.04). Occupational exposure to chemical products including organic solvents or petroleum showed a significant association with increased risk (OR = 8.05, 95% CI = 1.01-64.45). Medical histories of operation for appendicitis and blood transfusion were associated with decreased risk, but not significantly. Our results suggested that occupational exposure to chemicals might play a role in the risk of MM.

Profil biologiques compares du myelome multiple de l'adulte jeune (60ans) et de l'adulte age (60 ans)

L'etude retrospective comparative des aspects biologiques du myelome multiple chez 12 patients de plus de 60 ans et chez 19 patients de moins de 60 ans realisee au service d'hematologie clinique du C.H.U. de Yopougon de septembre 2003 a fevrier 2004 a donne les conclusions suivantes: AU PLAN EPIDEMIOLOGIQUE : L'age moyen est de 55;58 ans avec des extremes de 40 a 75 ans; un sex-ratio de 4 hommes pour I femme chez les malades de moins de 60 ans et 2 femmes pour 1 homme chez les patients de plus de 60 ans. Le groupe ethnique Kwa semble le plus touche. La plupart des patients resident a Abidjan avec un niveau socio-economique faible. La profession n'a pas d'influence sur la maladie. AU PLAN BIOLOGIQUE : Concernant le bilan hematologique; la plasmocytose medullaire est elevee (moyenne de 33;38pour cent) avec une legere accentuation chez les malades de moins de 60 ans (36;4pour cent). L'anemie avec un taux d'hemoglobine inferieur a 8.5 g/dl est generalement observee dans les deux groupes de notre serie et une thrombopenie chez 50pour cent des patients de notre serie. Concernant le bilan biochimique; la protidemie est elevee dans notre serie (moyenne de 106 g/fl. A l'electrophorese des protides; le pic monoclonal dans la zone des gamma globuline est generalement constante dans notre serie de meme que l'hypoalbuminemie qui est cependant tres severe chez les patients de plus de 60 ans. L'hypercalcemie et la fonction renale ne sont pas influencees par l'age. Le syndrome tumoral est tres important et s'accompagne d'une hyperactivite intrinseque chez l'adulte jeune caracterise par une elevation de la beta2 microglobuline et la CRP. Concernant le bilan immunologique; le myelome a lgG est predominant dans notre serie quel que soit le groupe d'age. La plupart des patients presentent un myelome a chaine legere kappa. Le taux d'IgG monoclonale ne semble pas etre influence par l'age et la baisse des Ig normales est constante dans les deux groupes d'age. AU NIVEAU PRONOSTIQUE : Le pronostic est generalement mauvais selon la classification de Dune et Salmon (stade III) dans les deux groupes. Il est moyen selon la classification de la SWOG; par contre la classification de KLEIN revele un pronostic sombre (groupe III) chez les patients de moins de 60 ans faisant ainsi de l'age jeune un facteur de mauvais pronostic

Valor pronóstico de la resonancia magnética en pacientes con mieloma múltiple: un estudio prospectivo / MRI prognostic value in multiple myeloma

Propósito: correlacionar los patrones de infiltración de la médula ósea (MO) por RM en pacientes con MM no tratados y valorar los resultados en conjunto con otros factores pronósticos utilizados (clínica, laboratorio, radiología). Material y métodos: evaluamos 21 pacientes con MM no tratados por RM y osteograma corporal para valorar lesiones óseas, plasmocitomas u osteoporosis. Resultados: están descriptos 3 patrones de infiltración de la MO por RM en éstos pacientes: focal (PF), difuso (PD) y variegado (PV). A diferencia de otras series, observamos estudios por RM con 2 o más patrones descriptos: 13 pacientes. Tentativamente proponemos denominar a ésta variedad de presentación, patrón mixto (PM). Conclusión: la RM es más sensible que la radiología convencional en la detección de lesiones óseas. Los patrones de infiltración de la MO por RM, combinados con el resto de factores convencionales usados, constituyen una herramienta muy útil en la determinación del pronóstico de la enfermedad. En nuestra serie, el PD, ya sea aislado o combinado con otros patrones (PM), correlacionó con factores pronósticos más severos

Detección del herpes virus humano 8 en pacientes con mieloma múltiple / HHV8 in patients with multiple myeloma

Objetivos: Evaluar la presencia del herpes virus en 8 pacientes con mieloma múltiple y en pacientes control, y en otras enfermedades hematológicas. Secundariamente determinar su relación con factores pronósticos y con la respuesta al tratamiento. Materiales y Métodos: Se incluirán 20 pacientes con diagnóstico de mieloma múltiple y 40 pacientes control. Se determinará en las biopsias de médula ósea al patrón y porcentaje de infiltración por células neoplásicas y el grado de angiogénesis. Se evaluará la presencia del virus en las biopsias y aspirados de médula ósea a través del método de PCR, al igual que en muestras de sangre periférica, donde también se realizará la determinación de anticuerpos contra el virus. En las biopsias se realizará hibridación in situ e inmunomarcación para caracterizar la estirpe celular de la población positiva. Resultados: Se han incorporado 28 pacientes; 13 tienen diagnóstico de mieloma múltiple y 15 pertenecen al grupo control. En las muestras de aspirado de médula ósea se realizó la búsqueda del ADN viral por la técnica de PCR sin encontrarse hasta el momento resultados positivos. Conclusiones: El estudio continúa en el período de recolección de datos; los resultados obtenidos del análisis por PCR en los aspirados de médula ósea concuerdan con la bibliografía internacional.

Tumours and tumour-like lesions of bone. The UHWI experience. University Hospital of the West Indies

A review of all primary tumours and tumour-like lesions of bone diagnosed in patients who attended the University Hospital of the West Indies over a 10-year period revealed 136 cases comprising 69 benign and 54 malignant tumours, and 13 cases of tumour-like lesions. The prevalence and clinical characteristics of the various lesions are discussed and compared with the findings in other series. The results of this analysis provide demographic data useful in the differential diagnosis of bone lesions locally.

Mieloma múltiplo: um perfil de 22 casos / Multiple myeloma: a profile of 22 cases

Os autores analisam 22 casos diagnosticados de mieloma múltiplo. A idade média dos pacientes por ocasiäo do diagnóstico foi de 58 anos, sendo 12 pacients dosexo masculino e 10 do sexo feminino. Em relaçäo ao quadro clínico, todos apresentavam queixas de dores ósseas, cujas localizaçöes mais freqüentes foram coluna vertebral e membros. Em 31,81% dos pacientes foi relatada perda de peso. As hemorragias, de maneira geral, näo foram freqüentes, estando presentes em 18,18% dos casos. Observou-se cisceromegalias em 31,81% dos casos; destes, todos apresentavam hepatomegalia. A anemia foi freqüente, estando presente em 86,36%. Com relaçäo ao estadiamento clínico, 90,90% dos casos encontravam-se no estádio III e imunoglobulina mais freqüentemente envolvida foi IgG