Huge Sellar Plasmacytoma as Differential Diagnosis of Invasive Pituitary Adenoma: A Case Report

Sellar plasmacytomas are rare tumors arising from plasma cells. They are often misdiagnosed as adenomas.We report the case of a 63-year-old woman with headache, cranial nerve III palsy and decreased visual acuity. Imaging revealed an extensive lesion centered on the clivus, extending to the cavernous sinus bilaterally and into the sphenoid sinus. The hormonal tests were compatible with panhypopituitarism and mild hyperprolactinemia. The first hypothesis was invasive pituitary adenoma. Partial resection was achieved, and the immunohistochemical evaluation was compatible with plasmacytoma. After a few weeks, she developed lumbar and hip pain, and the imaging confirming osteolytic lesions. The final diagnosis was multiple myeloma.

Calidad de vida y balance sagital en la evolución de los pacientes con mieloma múltiple tratados con vertebroplastia percutánea / Quality of Life and Sagittal Balance in the Evolution of Patients with Multiple Myeloma Who Received Treatment

RESUMEN Introducción: El mieloma múltiple representa el 1 por ciento de las neoplasias y el 10 por ciento de las hemopatías malignas. La edad media de presentación es 65 años. El dolor óseo en columna vertebral y costillas constituye su manifestación inicial. El tratamiento quirúrgico del mieloma múltiple en la columna consiste en descompresión amplia y artrodesis. La vertebroplastia se considera técnica de elección porque restablece el balance sagital y coronal, contribuye al fortalecimiento del cuerpo vertebral, y disminuye el dolor asociado a fracturas. Objetivo: Evaluar el efecto de la vertebroplastia sobre la calidad de vida y el balance sagital, en la disminución del dolor. Métodos: Se estudiaron 192 pacientes con mieloma múltiple con fractura en la columna, 80 tratados con vertebroplastia y 112 en forma conservadora. Se utilizó el cuestionario de Oswestry y la escala de Karnosfky. Resultados: La vertebroplastia disminuyó 0,52 veces el uso de analgésicos opioides. Conclusiones: La vertebroplastia representa un mayor beneficio de la calidad de vida de los pacientes aquejados de mieloma múltiple con fractura vertebral; repercute en el mejoramiento del balance sagital, el nivel de discapacidad y la capacidad para realizar tareas cotidianas(AU)

ABSTRACT Introduction: Multiple myeloma accounts for 1 percent of neoplasms and for 10 percent of malignant hematomas. The mean onset age is 65 years. Bone pain in the spine and ribs is its initial manifestation. Surgical treatment for multiple myeloma in the spine consists in wide decompression and arthrodesis. Vertebroplasty is considered the choice technique because it restores sagittal and coronal balance, contributes to the strengthening of the vertebral body, and reduces pain associated with fractures. Objective: To assess the effect of vertebroplasty on quality of life and the effect of sagittal balance in reducing pain. Methods: 192 patients with multiple myeloma and spinal fracture were studied, 80 treated with vertebroplasty and 112 treated conservatively. The Oswestry questionnaire and the Karnofsky scale were used. Results: Vertebroplasty decreased the use of opioid analgesics by 0.52 times. Conclusions: Vertebroplasty represents a greater benefit for the quality of life of patients suffering from multiple myeloma with vertebral fracture. It affects the improvement of sagittal balance, the level of disability, and the ability to perform daily tasks(AU)

Evaluation of nutritional risk factors in hematopoietic stem cell transplantation-eligible patients / Avaliação dos fatores de riscos nutricionais em pacientes pré-transplante de célula-tronco hematopoiética

ABSTRACT Objective To evaluate the nutritional risk factors in patients eligible for hematopoietic stem cell transplantation. Methods A cross-sectional, descriptive study conducted with patients recruited from an hematology outpatient clinic. Study variables included demographic and clinical data, patient-generated global subjective assessment findings, anthropometric indicators, food intake and oxidative stress levels. The level of significance was set at 5% (p<0.05). Results The sample comprised 72 patients, mean age of 48.93 years (14.5%). Multiple myeloma was the most prevalent condition (51.4%) in this sample. Most patients (55.6%) were overweight according to body mass index and at risk of cardiovascular disease according to waist circumference, conicity index and percentage of body fat. Sarcopenia was associated with risk of cardiovascular disease, hip-to-waist ratio (p=0.021), muscle strength depletion (p<0.001), food intake (p=0.023), reduced functional capacity (p=0.048), self-reported well-nourished status; p=0.044) and inadequate vitamin B6 (p=0.022) and manganese (p=0.026) intake. Elevated oxidative stress, detected in 33.3% of patients in this sample, was not associated with sarcopenia. Conclusion Most patients in this sample were overweight and sarcopenic. Lean mass depletion was associated with risk of cardiovascular disease, reduced muscle strength, food intake changes, reduced functional capacity, self-reported well-nourished status and inadequate intake of vitamin B6 and manganese, but not with oxidative stress.

RESUMO Objetivo Avaliar os fatores de riscos nutricionais em pacientes pré-transplante de célula-tronco hematopoiética. Métodos Estudo transversal, descritivo, realizado com pacientes de um ambulatório de hematologia. As variáveis estudadas foram demográficas, dados clínicos, avaliação subjetiva global produzida pelo próprio paciente, indicadores antropométricos, ingestão alimentar e estresse oxidativo. Os dados foram considerados estatisticamente significativos quando p<0,05. Resultados A amostra do estudo foi constituída por 72 pacientes, com média de idade de 48,93 (14,5%) anos e com mieloma múltiplo (51,4%) como a patologia mais prevalente. Conforme índice de massa corporal, 55,6% dos pacientes encontravam-se com excesso de peso. De acordo com a circunferência da cintura, índice de conicidade e percentual de gordura corporal, houve prevalência de risco para doença cardiovascular. A sarcopenia foi associada ao risco de doença cardiovascular pela relação cintura/quadril (p=0,021), depleção da força muscular (p<0,001), além da ingestão alimentar (p=0,023), da capacidade funcional reduzida (p=0,048) e do diagnóstico de "bem nutrido" (p=0,044), conforme a avaliação subjetiva global, e com consumo inadequado de vitamina B6 (p=0,022) e de manganês (p=0,026). Dentre os avaliados, 33,3% apresentaram estresse oxidativo elevado sem associação com sarcopenia. Conclusão Pacientes do pré-transplante se apresentam, em sua maioria, com excesso de peso, mas com sarcopenia, estando essa ausência de massa magra associada a risco de doença cardiovascular, depleção da força muscular, alteração da ingestão alimentar, redução da capacidade funcional, classificação de "bem nutrido", segundo a avaliação subjetiva global e consumo inadequado de vitamina B6 e manganês, não estando associada a estresse oxidativo.

Cardiovascular risk and use of conicity index in patients submitted to autologous hematopoietic stem cell transplantation / Risco cardiovascular e uso do índice de conicidade em pacientes submetidos ao transplante autólogo de células-tronco hematopoiéticas

ABSTRACT Objective To analyze the prevalence of overweight and the use of conicity index for cardiovascular risk assessment in individuals submitted to autologous hematopoietic stem cell transplantation. Methods The sample comprised 91 patients of both sexes, who underwent autologous hematopoietic stem cell transplantation from September 2008 to December 2013, aged 18 years or over. To determine the nutritional profile, we collected anthropometric data on weight, height, waist circumference upon hospital admission. The body mass index and the conicity index were calculated. Results A total of 91 patients diagnosed with multiple myeloma, Hodgkin's lymphoma, non-Hodgkin's lymphoma and other conditions were evaluated. The mean age was 43.5 (14.2) years, 63.7% were male. We verified that according to the body mass index, 63.7% were overweight and, according to waist circumference, 74.7% had a higher risk for cardiovascular diseases. According to the calculation of the conicity index, 92.3% of patients presented increased cardiovascular risk. Patients with multiple myeloma had a higher conicity index when compared to other patients (p<0.01). Conclusion This study revealed a high prevalence of overweight and cardiovascular risk. It should be noted that the conicity index was a good method to evaluate cardiovascular risk and that new studies using this index should be performed.

RESUMO Objetivo Analisar a prevalência de excesso de peso e a utilização do índice de conicidade para a avaliação do risco cardiovascular em indivíduos submetidos ao transplante autólogo de células-tronco hematopoiéticas. Métodos A amostra foi composta por 91 pacientes, de ambos os sexos, submetidos ao transplante autólogo de células-tronco hematopoiéticas, de setembro de 2008 a dezembro de 2013, com idade igual ou superior a 18 anos. Para traçar o perfil nutricional, foram coletados dados antropométricos, como peso, altura e circunferência da cintura, na admissão hospitalar. Calcularam-se o índice de massa corporal e o índice de conicidade. Resultados Foram avaliados 91 pacientes, diagnosticados com mieloma múltiplo, linfoma de Hodgkin, linfoma não Hodgkin e outras patologias. A média de idade foi de 43,5 (14,2) anos, e 63,7% eram do sexo masculino. Verificou-se que, de acordo com o índice de massa corporal, 63,7% apresentavam excesso de peso e, segundo a circunferência da cintura, 74,7% possuíam maior risco para doenças cardiovasculares. De acordo com o cálculo do índice de conicidade, constatou-se que 92,3% dos pacientes apresentavam risco cardiovascular aumentado. Os pacientes com mieloma múltiplo apresentaram índice de conicidade mais elevado, se comparado aos outros pacientes (p<0,01). Conclusão Este estudo revelou alta prevalência de excesso de peso e de risco cardiovascular. Salienta-se que o índice de conicidade se mostrou um bom método para avaliação do risco cardiovascular e que novos estudos utilizando este índice devem ser realizados.

The need for endodontic treatment and systemic characteristics of hematopoietic stem cell transplantation patients

Abstract The aim of this study is to investigate the relationship between the epidemiological and clinical profiles of patients before and after hematopoietic stem cell transplantation (HSCT) and the need for endodontic treatment. The subjects included 188 individuals enrolled in the dental care program for transplanted patients of the School of Dentistry, Federal University of Minas Gerais (Faculdade de Odontologia da Universidade Federal de Minas Gerais, FO-UFMG) from March 2011 through March 2016. The patients were subjected to an HSCT conditioning dental regimen based on a thorough clinical and radiographic evaluation. Intraoral periapical and bite-wing X-rays were obtained, and after evaluation, specific dental treatment was planned and performed. The following demographic and clinical data were collected from the patients' medical records: age, gender, transplantation stage, primary disease, transplant type, medication used, complete blood count at the time of visit, and need for endodontic treatment. The Kolmogorov-Smirnov and the chi-square tests were used. Leukemia (31.3%) and multiple myeloma (17.9%) were the most prevalent primary diseases. Most patients were subjected to allogeneic-related transplantation (83.6%). Most patients exhibited platelet counts and hemoglobin concentrations below the reference values in the pre-transplantation stage, while the neutrophil and platelet counts and the hemoglobin levels were within the reference ranges in the post-transplantation stage. The proportions of individuals requiring endodontic treatment were similar between the pre- and post-transplantation groups: 24.3% and 24.7%, respectively. The systemic conditions of the patients referred for dental treatment were compromised.

Plasmocitoma extramedular, intracanal extradural, posterior a artrodesis cervical en un paciente con mieloma múltiple / Extramedullary, intracanal epidural plasmacytoma after cervical arthrodesis in a patient with multiple myeloma

El plasmocitoma extradural a nivel cervical es una de las patologías menos frecuentes dentro de los tumores extramedulares del mieloma múltiple; así mismo, es infrecuente que cause compresión medular o de las raíces nerviosas. Se presenta este caso por su gravedad y baja frecuencia en un paciente con diagnóstico de mieloma múltiple en tratamiento, que concurre con síntomas neurológicos (parestesia y paresia braquial bilateral) en la semana posterior a la cirugía de liberación meningorradicular y artrodesis cervical (C6-C7), en el mismo nivel de aparición de un plasmocitoma intracanal, extradural diagnosticado por resonancia magnética y confirmado con el resultado de la biopsia intraquirúrgica. Se ha publicado un caso similar al presentado, pero con la diferencia de que el paciente debutó con cuadriparesia. Se conoce también un caso de plasmocitoma originado en la médula espinal de la región torácica. En nuestro paciente, al igual que en un caso reportado con mieloma múltiple asintomático con manifestación primaria de plasmocitoma extramedular, la evolución de la enfermedad fue rápida, y el paciente falleció en menos de un año desde el diagnóstico. Nivel de Evidencia: IV.

Epidural plasmacytoma at the cervical spine is one of the less common extramedullary tumors in multiple myeloma. Likewise compression of the spinal cord or the nerve roots is uncommon. The following case is presented due to its rarity and severity. A patient diagnosed with and being treated for multiple myeloma presented with neurological symptoms (paresthesia and bilateral upper extremity weakness) a week after decompression and C6-C7 fusion, at the same level of an intracanal, epidural plasmacytoma detected by magnetic resonance and confirmed by intraoperative biopsy. A similar case was published, however it differs in that the patient presented with quadriplegia. There is also a reported case of plasmacytoma originating in the thoracic region of the spinal cord. In our patient, similar to the previously reported case of asymptomatic multiple myeloma with primary manifestation of extramedullary plasmacytoma, progression of the disease was rapid and the patient died less than one year from diagnosis. Level of Evidence: IV.

Relevance of prognostic factors in the decision-making of stem cell transplantation in Myelodysplastic Syndromes / Relevância dos fatores prognósticos na decisão do transplante de células-tronco na Síndrome Mielodisplásica

ABSTRACT The hematopoietic stem cell transplantation (HSCT) is the only curative alternative for Myelodysplastic Syndrome (MDS), but many patients are not eligible for this treatment, as there are several limiting factors, especially in the case of patients with low-risk MDS. The aim of this study is to discuss the factors that can guide the decision-making on referring or not a patient to HSCT. Three cases of MDS, two of which were submitted to HSCT are presented. We intend to report the difficulties in referring patients with MDS to transplant and the prognostic factors that contribute to define eligibility.

RESUMO O transplante de células-tronco hematopoéticas (TCTH) é a única alternativa curativa para Síndrome Mielodisplásica (SMD), porém muitos pacientes não são elegíveis para esta opção, pois existem diversos fatores limitantes, principalmente no caso de pacientes com SMD de baixo risco. O objetivo do estudo é discutir os fatores que podem orientar a decisão no encaminhamento ou não para o TCTH. São apresentados três casos de SMD, dos quais dois foram submetidos ao TCTH. Nos propomos a relatar as dificuldades no encaminhamento dos pacientes com SMD ao transplante e os fatores prognósticos que contribuem para definir a elegibilidade.

Association of oxidative stress and DNA damage with grafting time in patients with multiple myeloma and lymphoma submitted to autologous hematopoietic stem cell transplantation / Associação do estresse oxidativo e dano ao DNA com o tempo de enxertia em pacientes com mieloma múltiplo e linfomas submetidos a transplante autólogo de células-tronco hematopoéticas

ABSTRACT The aim of the study was to investigate the association between oxidative stress and DNA damage with grafting time in patients submitted to autologous hematopoietic stem-cell transplantation (HSCT). The study included 37 patients submitted to autologous HSCT diagnosed with Multiple Myeloma (MM) and lymphoma (Hodgkin’s and non-Hodgkin’s). Biomarkers of oxidative stress and DNA damage index (DI) were performed at baseline (pre-CR) of the disease and during the conditioning regimen (CR), one day after the HSCT, ten days after HSCT and twenty days after HSCT, as well as in the control group consisting of 30 healthy individuals. The outcomes showed that both groups of patients had an hyperoxidative state with high DI when compared to baseline and to the control group and that the CR exacerbated this condition. However, after the follow-up period of the study, this picture was re-established to the baseline levels of each pathology. The study patients with MM showed a mean grafting time of 10.75 days (8 to 13 days), with 10.15 days (8 to 15 days) for the lymphoma patients. In patients with MM, there was a negative correlation between the grafting time and the basal levels of GPx (r = -0.54; p = 0.034), indicating that lower levels of this important enzyme are associated with a longer grafting time. For the DI, the correlation was a positive one (r = 0.529; p = 0.030). In the group with lymphoma, it was observed that the basal levels of NOx were positively correlated with grafting time (r = 0.4664, p = 0.032). The data indicate the potential of these biomarkers as predictors of toxicity and grafting time in patients with MM and Lymphomas submitted to autologous HSCT.

RESUMO O objetivo do estudo foi investigar a associação entre estresse oxidativo e dano ao DNA com o tempo de enxertia em pacientes submetidos ao transplante de células-tronco hematopoéticas autólogo (TCTH). Participaram do estudo 37 pacientes submetidos ao TCTH autólogo com diagnóstico de mieloma múltiplo (MM) e Linfomas (Hodgkin e não Hodgkin). Biomarcadores de estresse oxidativo e índice de dano ao DNA (ID) foram determinados no estado basal (Pré-RC) das doenças e durante o regime de condicionamento (RC), um dia após o TCTH, dez dias após o TCTH e vinte dias após o TCTH e no grupo controle composto por 30 individuos saudáveis. Os resultados demonstraram que os dois grupos de pacientes apresentaram um estado hiperoxidativo com elevado ID quando comparados ao estado basal e ao grupo controle e que o RC exacerbou essa condição. No entanto, após o tempo de acompanhamento do estudo, esse quadro foi reestabelecido ao estado basal de cada patologia. Os pacientes do estudo com MM apresentaram uma média do tempo de enxertia de 10,75 dias (8 a 13 dias), e de 10,15 dias (8 a 15 dias) para o grupo Linfoma. Nos pacientes com MM houve uma correlação negativa entre o tempo de enxertia e os níveis basais de GPx (r=-0,54; p=0,034), indicando que níveis mais baixos de GPx estão relacionados a um maior tempo de enxertia, e para o ID, a correlação foi positiva (r=0,529; p=0,030). No grupo com Linfoma, observou-se que os níveis basais de NOx correlacionaram-se positivamente com o tempo de enxertia (r= 0,4664; p=0,032). Os dados apontam para o potencial desses biomarcadores como preditores da toxicidade e do tempo de enxertia em pacientes com MM e Linfomas submetidos ao TCTH autólogo

Are the binary typology models of alcoholism valid in polydrug abusers ?

Objective: To evaluate the dichotomy of type I/II and type A/B alcoholism typologies in opiate-dependent patients with a comorbid alcohol dependence problem (ODP-AP). Methods: The validity assessment process comprised the information regarding the history of alcohol use (internal validity), cognitive-behavioral variables regarding substance use (external validity), and indicators of treatment during 6-month follow-up (predictive validity). Results: ODP-AP subjects classified as type II/B presented an early and much more severe drinking problem and a worse clinical prognosis when considering opiate treatment variables as compared with ODP-AP subjects defined as type I/A. Furthermore, type II/B patients endorse more general positive beliefs and expectancies related to the effect of alcohol and tend to drink heavily across several intra- and interpersonal situations as compared with type I/A patients. Conclusions: These findings confirm two different forms of alcohol dependence, recognized as a low-severity/vulnerability subgroup and a high-severity/vulnerability subgroup, in an opiate-dependent population with a lifetime diagnosis of alcohol dependence. .

Experiencia de 22 años de trasplante autólogo de células hematopoyéticas en pacientes con mieloma múltiple o amiloidosis sistémica. 1992-2014 / Autologous hematopoietic cell transplantation in patients with multiple myeloma. Experience in 53 patients

Background: Autologous hematopoietic cell transplantation (THA) in patients with multiple myeloma and amyloidosis is the standard of care to promote disease free survival and quality of life. Aim: To report our experience with THA in patients with multiple myeloma. Material and Methods: Retrospective review of the hematopoietic cell transplantation database of a hospital of a Medical School. Forty seven patients with multiple myeloma and six with amyloid light chain amyloidosis were identified. Clinical and demographic data were obtained from the records. Results: The overall five year survival of patients was 55%. Transplant-related or non-relapse mortality occurred in 7%. We found no differences in outcomes among patients younger or older than 50 years. Conclusions: Our data supports that THA can be done in our country with similar results to those obtained in international transplantation centers. Chronological age should not be a limitation to offer this therapy to patients with multiple myeloma and amyloidosis.

Autologous stem cell transplantation improves quality of life in economically challenged, brazilian multiple myeloma patients

OBJECTIVES: 1) To characterize the impact of multiple myeloma on the quality of life of patients treated in two public institutions in São Paulo State, Brazil, using a generic Short Form 36 Health Survey and a questionnaire specific for oncologic patients (QLQ-C30) upon diagnosis, after the clinical treatment, and at day +100 after autologous stem cell transplantation; 2) to evaluate whether autologous stem cell transplantation can improve the quality of life of our economically challenged population aside from providing a clinical benefit and disease control. METHODS: We evaluated 49 patients with multiple myeloma (a total of 70 interviews) using the two questionnaires. The scores upon diagnosis, post-treatment/pre-autologous stem cell transplantation, and at D+100 were compared using ANOVA (a comparison of the three groups), post hoc tests (two-by-two comparisons of the three groups), and paired t-tests (the same case at two different times). RESULTS: Of the included patients, 87.8 percent had a family budget under US $600 (economic class C, D, or E) per month. The generic Short Form 36 Health Survey questionnaire demonstrated that physical function, role-physical, and bodily pain indices were statistically different across all three groups, favoring the D+100 autologous stem cell transplantation group (ANOVA). The questionnaire specific for oncologic patients, the QLQ-C30 questionnaire, confirmed what had been demonstrated by the Short Form 36 Health Survey with respect to physical function and bodily pain, with improvements in role functioning, fatigue, and lack of appetite and constipation, favoring the D+100 autologous stem cell transplant group (ANOVA). The post hoc tests and paired t-tests confirmed a better outcome after autologous stem cell transplantation CONCLUSION: The questionnaire specific for cancer patients seems to be more informative than the generic Short Form 36 Health Survey questionnaire and reflects the real benefit of autologous stem cell transplantation in the quality of life of multiple myeloma patients in two public Brazilian institutions that provide assistance for economically challenged patients.

Serological monitoring of a Toxoplasma infection after hematopoietic stem cell transplantation / Monitoramento sorológico de uma infecção toxoplásmica após transplante de células progenitoras hematopoiéticas

We report a primary response to Toxoplasma gondii following a hematopoietic stem cell transplantation in a patient with multiple myeloma. The primary response to T. gondii was supported by IgM, IgG and IgA seroconversion. The patient was promptly treated and there were no complications related to toxoplasmosis in the subsequent months.

Esse relato de caso descreve uma resposta primária ao Toxoplasma gondii após transplante de células progenitoras hematopoiéticas em paciente com mieloma múltiplo. A resposta primária para o T. gondii foi evidenciada pela soroconversão observada na resposta de anticorpos IgM, IgG e IgA. O paciente foi prontamente tratado e complicações relacionadas à toxoplasmose não foram observadas nos meses subseqüentes.

Prevalence of chimerism after non-myeloablative hematopoietic stem cell transplantation / Prevalência do quimerismo após transplante de células hematopoiéticas não-mieloablativo

Context and objective: Non-myeloablative hematopoietic stem cell transplantation (NMA-HSCT) is performed in onco-hematological patients who cannot tolerate ablative conditioning because of older age or comorbidities. This approach does not completely eliminate host cells and initially results in mixed chimerism. Long-term persistence of mixed chimerism results in graft rejection and relapse. Involvement of graft-versus-host disease is concomitant with complete chimerism and graft-versus-tumor effect. The aim of this study was to evaluate the prevalence of chimerism in onco-hematological patients who underwent NMA-HSCT. Desingn and setting: Observational clinical study on chimerism status after human leukocyte antigen-identical NMA-HSCT at the Discipline of Hematology and Hemotherapy of Universidade Federal de São Paulo. Methods: We sequentially analyzed the amplification of APO-B, D1S80, DxS52, FVW, 33.6, YNZ-2 and H-ras primers using variable number of tandem repeats (VNTR) on 17 pairs and fluorescent in situ hybridization (FISH) with the XY probe and SRY primer on 13 sex-unmatched pairs. RESULTS: The informativeness of the primers using VNTR was 60 percent for APO-B, 75 percent D1S80, 36 percent DxS52, 14 percent FVW, 40 percent YNZ-22 and 16 percent H-ras. The SRY primer was informative in female receptors with male donors. The XY-FISH method was informative in 100 percent of the sex-unmatched pairs. Conclusion: These methods were sensitive and informative. In VNTR, the association of APO-B with D1S80 showed 88 percent informativeness. The quantitative FISH method was more sensitive, but had the disadvantage of only being used for sex-unmatched pairs.

Contexto e objetivo: O transplante de células hematopoiéticas não-mieloablativo é realizado em pacientes com doenças onco-hematológicas que não suportam condicionamentos ablativos devido à elevada idade ou ao acometimento por comorbidades. Esta abordagem não elimina completamente as células do hospedeiro, resultando, inicialmente, em quimerismo misto. A persistência do quimerismo misto na evolução de longo prazo resulta na rejeição ao enxerto e recaída. O acometimento pela doença do enxerto contra hospedeiro é concomitante ao quimerismo completo e ao efeito enxerto versus tumor. O objetivo deste estudo foi avaliar a prevalência do quimerismo em doenças onco-hematológicas tratadas com o transplante não-mieloablativo de células hematopoiéticas. Tipo de estudo e local: Estudo clínico observacional do estado de quimerismo após transplante antígenos leucocitários humanos-idêntico não-mieloabaltivo realizado na Disciplina de Hematologia e Hemoterapia da Universidade Federal de São Paulo. Métodos: Analisamos sequencialmente a amplificação dos primers APO-B, D1S80, DxS52, FVW, 33,6, YNZ-22, H-ras pelo VNTR (variable number of tandem repeats) em 17 pares e FISH (fluorescent in situ hybridization) pela sonda XY e do primer SRY em 13 pares de não relacionados a sexo. Resultado: A informatividade dos primers pelo VNTR foi de 60 por cento para APO-B; 75 por cento D1S80; 36 por cento DxS52; 14 por cento FVW; 40 por cento YNZ-22 e 16 por cento H-ras. O primer SRY foi informativo em receptores femininos com doadores masculinos. O método XY-FISH foi informativo em 100 por cento dos pares de não relacionado a sexo. Conclusão: Estes métodos foram sensíveis e informativos. No VNTR, a associação do APO-B com D1S80 mostrou 88 por cento de informatividade. O FISH, método quantitativo, foi mais sensível, porém com desvantagem de ser usado somente nos pares não relacionados a sexo.

Mieloma múltiplo da coluna: avaliação do tratamento cirúrgico / Mieloma múltiple de la columna: evaluacióndel tratamiento quirúrgico / Multiple myeloma of the spine: surgical treatment evaluation

INTRODUÇÃO: o mieloma múltiplo é uma neoplasia maligna de proliferação monoclonal e difusa de células plasmáticas na medula óssea comum no sistema esquelético. O sítio mais frequente é a coluna vertebral. O mieloma múltiplo pode provocar dor intratável, instabilidade e déficit neurológico na coluna vertebral. O tratamento cirúrgico dessa neoplasia na coluna vertebral consiste em descompressão ampla e artrodese com ou sem instrumentação. A literatura médica apresenta poucas pesquisas com foco no resultado clínico em relação à dor e à melhora neurológica. OBJETIVO: avaliar os resultados clínicos do tratamento cirúrgico de pacientes portadores de mieloma múltiplo na coluna. MÉTODOS: estudo retrospectivo de 16 pacientes portadores de mieloma múltiplo na coluna que foram submetidos à descompressão cirúrgica com ou sem instrumentação. A extensão das lesões foi classificada de acordo com o método de Tomita. Os pacientes foram avaliados em relação à melhora dos sintomas dolorosos e em relação ao quadro neurológico de acordo com a classificação de Frankel. RESULTADOS: os quatro pacientes que apresentavam exame normal (Frankel E) permaneceram sem déficit neurológico no período pós-operatório. Dois pacientes Frankel D evoluíram para Frankel E. Dos três pacientes que apresentavam Frankel C, dois obtiveram melhora (Frankel D) e um manteve o mesmo grau de déficit. Os quatro pacientes que apresentavam Frankel B evoluíram para Frankel D após a descompressão. Déficit neurológico completo (Frankel A) estava presente em três pacientes, dois evoluíram com melhora da função neurológica (Frankel B e C) e um evoluiu a óbito no pós-operatório imediato. CONCLUSÕES: o tratamento cirúrgico do mieloma na coluna apresenta bons resultados em relação à dor e melhora do quadro neurológico em casos bem selecionados.

INTRODUCTION: multiple myeloma is a malignant neoplasm of monoclonal and diffuse proliferation of plasma cells that typically involves the bone marrow. The spine is one of the most commonly affected sites. It may cause severe intractable pain, spinal instability, or neurological complications. The surgical treatment for multiple myeloma includes open decompressive surgery and arthrodesis with or without stabilization. The medical literature presents little researches focused on clinical outcome with regard to pain and neurological improvement. OBJECTIVE: to evaluate clinical outcomes in patients surgically treated for multiple myeloma of the spine. METHODS: a total of 16 consecutive patients who underwent spinal column decompression for multiple myeloma with or without stabilization were retrospectively studied. The local extent of tumor was graded based on Tomita et al. classification. The patients were assessed with regard to pain and neurological improvement according to Frankel's classification. RESULTS: the four patients with normal examination (Frankel E) remained without neurologic deficit in the postoperative period. Two Frankel D patients evolved to Frankel E. Two Frankel C patients evolved to improvement (Frankel D) and one Frankel C patient remained with the same status. Four Frankel B patients evolved to Frankel D after decompression. Two Frankel A patients (complete neurological deficit) also improved their neurological status (Frankel B and C), and one died in the immediate postoperative period. CONCLUSIONS: surgical treatment of spinal lesions due to myeloma presents good clinical outcome based on pain and neurological improvement in well selected cases.

INTRODUCCIÓN: el mieloma múltiple es una neoplasia maligna de proliferación monoclonal y difusa de células plasmáticas en la médula ósea siendo común en el sistema esquelético. El sitio más frecuente es la columna vertebral. El mieloma múltiple puede provocar dolor intratable, inestabilidad y déficit neurológico en la columna vertebral. El tratamiento quirúrgico del mieloma en la columna vertebral consiste en descompresión amplia y artrodesis con o sin instrumentación. La literatura médica presenta pocas investigaciones con enfoque en el resultado clínico en relación al dolor y mejoría neurológica. OBJETIVOS: evaluar los resultados clínicos del tratamiento quirúrgico de pacientes portadores del mieloma múltiple en columna. MÉTODOS: estudio retrospectivo de 16 pacientes portadores del mieloma múltiple en la columna que fueron sometidos a la descompresión quirúrgica con o sin instrumentación. La extensión de las lesiones fue clasificada de acuerdo con el método de Tomita. Los pacientes fueron evaluados en relación a la mejoría de los síntomas dolorosos y en relación al cuadro neurológico de acuerdo con la clasificación de Frankel. RESULTADOS: los cuatro pacientes que presentaron examen normal (Frankel E) permanecieron sin déficit neurológico en el postoperatorio. Dos pacientes Frankel D evolucionaron para Frankel E. De los tres pacientes que presentaron Frankel C, dos obtuvieron mejoría (Frankel D) y uno mantuvo el mismo grado de déficit. Los cuatro pacientes que presentaron Frankel B evolucionaron para Frankel D después de la descompresión. Déficit neurológico completo (Frankel A) estaba presente en tres pacientes, dos evolucionaron con mejoría de la función neurológica (Frankel B y C) y un evolucionó con óbito en el postoperatorio inmediato. CONCLUSIÓN: el tratamiento quirúrgico del mieloma en la columna presenta buenos resultados en relación al dolor y mejoría del cuadro neurológico en casos bien seleccionados.

Compromiso pleural en la leucemia de células plasmáticas: reporte de un caso / Pleural effusion in plasma cell leukemia: a case report

Multiple myeloma is a low malignant, non-Hodgkin's lymphoma, which is characterized by infiltration of the bone marrow by clonal proliferation of atypical plasma cells. Extramedullary manifestations are relatively rare. Serous effusions in multiple myeloma are uncommon (6 percent of cases) but a myelomatous pleural effusion occurring in these patients is extremely rare. Plasma cell leukemia, occurring either de novo or in patients with long standing multiple myeloma, is the least common type of plasma cell dyscrasia. The authors describe the course of plasma cellular leukemia in a 63-year-old male patient where the first manifestation of the disease was a bacteremic pneumococcal pneumonia and a contralateral pleural exudate with a cytological finding of plasma cells. The exudate disappeared after the first cycle of chemotherapy (vincristine, adriamycin, dexamethasone). After the third cycle of chemotherapy remission of the disease was recorded which was, however, short. After three months' remission the disease recurred, consequently underwent autologous bone marrow transplantation. The patient has maintained complete remission for 12 months after diagnosis. Pleural effusion is an unusual but important complication of multiple myeloma and does not necessarily carry the grave prognosis implied in previous reports.

El mieloma múltiple es una neoplasia maligna de células plasmáticas que invade la médula ósea y otros tejidos. Las manifestaciones extramedulares son relativamente raras. El derrame pleural en el mieloma múltiple es poco frecuente (6 por ciento de los casos), y el derrame pleural neoplásico es extremadamente raro. La leucemia de células plasmáticas, que se produce de novo o en pacientes con mieloma múltiple, es la variedad menos común de discrasia de células plasmáticas. Los autores describen el cuadro clínico de un paciente de 63 años con leucemia de células plasmáticas, donde la primera manifestación de la enfermedad fue una neumonía neumocócica bacteriémica asociado a un derrame pleural contralateral, que correspondió a un exudado predominio mononuclear. El examen citológico reveló abundantes células plasmáticas inmaduras en el líquido pleural y la sangre periférica. El derrame pleural desapareció después del primer ciclo de quimioterapia (vincristina, adriamicina, dexametasona). Después de tres meses de remisión, la enfermedad neoplásica recidivó, siendo el paciente sometido a trasplante autólogo de médula ósea. El paciente se ha mantenido en remisión completa un año después del diagnóstico. El derrame pleural es una complicación poco común, pero importante, del mieloma múltiple y no necesariamente conlleva un mal pronóstico.

Plasmocitoma solitario de cabeza: presentación de dos casos

Se trata de un tumor de baja frecuencia constituido de células plasmáticas que pueden aparecer a nivel de cabeza y cuello. Estos constituyen menos del 1 por ciento de las neoplasias malignas de esta región. Deben ser diferenciados del mieloma múltiple, lo que puede ser dificultoso porque un variable porcentaje va asociado a un desarrollo más tardío de esta patología. Describimos dos casos de plasmocitoma solitario parietal. La clínica, diagnóstico y los problemas terapéuticos(radioterapia, cirugía o combinación de ambos) son discutidos y revisados en la literatura

Solitary plasmocytoma is a rare plasmactic cell tumor occurring in the head and neck. These constitute, less than 1% of all head and neck malignancies. They must be differentiated from multiple myeloma because a varying percentage may be associated at a later date with the development of multiple myeloma. We describe two cases of plasmocytomas, both in parietal bone. The clinical, diagnosis and therapeutic problems (radiation, surgery or a combination of both) are discussed and reviewed in the literature

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.

Tratamiento quirúrgico de las lesiones de la columna vertebral en pacientes con mieloma múltiple / Surgical treatment of the vertebral column injuries in patients with multiple myeloma

Se estudiaron 20 pacientes con diagnóstico de mieloma múltiple, 8 hombres y 12 mujeres, edad promedio de 54 años, operados por lesiones de la columna vertebral para evaluar los resultados del tratamiento quirúrgico. Se comprobó que el mieloma G y el Bence Jones fueron los más frecuentes (9 y 6 pacientes), y la mayoría se hallaba en estadio III o II de Durie-Salmon (11 y 8, respectivamente); la localización más frecuente fue la dorsal (14 casos), en 12 eran de tipo osteolítica y 7 tenían destrucción extensa y fracturas. Se realizó recanalización intralesional con estabilización interna en 17; en otros 2, estabilización sin recanalización, y en 1, laminectomía solamente. Hubo complicaciones en 13 casos, y de los 18 procesos reportados, la sepsis local, la neuritis posoperatoria y los granulomas de la herida fueron los más frecuentes. En el momento de la intervención se reportó dolor, intenso o incapacitante en 13 pacientes y en 18 había manifestaciones de daño neurológico. La evaluación al año de la operación mostró que 12 (60 por ciento) pacientes no tenían dolor o era ligero, y 14 (70 por ciento), no presentaban manifestaciones neurológicas. Se halló que 5 enfermos fallecieron, en el primer año de la intervención, por complicaciones de la enfermedad mielomatosa, no relacionadas con la cirugía; uno de los fallecidos presentó recidiva de la lesión mielomatosa de la columna. Se concluyó que el tratamiento quirúrgico de las lesiones de la columna vertebral en los enfermos con mieloma múltiple y manifestaciones dolorosas y/o neurológicas puede ser una posibilidad terapéutica beneficiosa