Encefalite herpética associada a mieloma múltiplo: desafio diagnóstico / Herpetic encephalitis associated with multiple myeloma: a diagnostic challenge

O mieloma múltiplo é a segunda neoplasia onco-hematológica mais comum, correspondendo a 1% das neoplasias malignas. Trata-se de uma condição subdiagnosticada, assim como a encefalite herpética. Entretanto, é comum a relação do mieloma com infecções, em decorrência do acometimento da imunidade humoral. A encefalite viral herpética tem como principal agente o vírus do herpes simples 1. O caso foi descrito baseado em um raciocínio clínico, visando contribuir para uma melhor caracterização do quadro clínico e do diagnóstico de duas entidades clínicas que possuem baixa suspeição diagnóstica e elevada morbimortalidade. Durante o curso da investigação, foram propostas variadas etiologias como responsáveis pelo rebaixamento do nível de consciência, levando a uma pesquisa de um espectro mais amplo de hipóteses diagnósticas, as quais precederam a confirmação do diagnóstico final. O quadro clínico atípico descrito apresentou obstáculos à suspeição diagnóstica correta, pois a ausência dos sinais e sintomas característicos de ambas as doenças levaram à pesquisa de um arsenal mais amplo de diagnósticos diferenciais. Assim, o atraso no diagnóstico e o início tardio do tratamento foram fatores que contribuíram para o prognóstico reservado do paciente. A associação dessas doenças é pouco descrita na literatura, de modo que mais estudos acerca do assunto se fazem necessários. (AU)

Multiple myeloma is the second most common onco-hematologic neoplasm, accounting for 1% of malignant neoplasms. As herpetic encephalitis, it is an underdiagnosed condition. However, the relation of myeloma with infections is common, due to the involvement of humoral immunity. Herpetic encephalitis has as its main etiological agent the herpes simplex virus 1. The case was described based on a clinical reasoning, aiming to contribute to a better characterization of the clinical picture and diagnosis of two entities that have low rates of diagnostic suspicion and high rates of morbidity and mortality. During the course of investigation, several etiologies were proposed as responsible for the decreased level of consciousness, leading to a search for a broader range of diagnostic hypotheses, which preceded confirmation of the final diagnosis. The atypical clinical picture described presented obstacles to the correct diagnostic suspicion, as the absence of symptoms and signs characteristic of both diseases led to the search for a broader arsenal of differential diagnoses. Thus, delayed diagnosis and late treatment were factors that contributed to the patient's reserved prognosis. The association of these diseases is poorly described in the literature, so further studies on that subject are required. (AU)

Artritis séptica de la rodilla como forma de presentación de mieloma múltiple / Septic Arthritis of the Knee as a Presentation of Multiple Myeloma

RESUMEN Introducción: El mieloma múltiple constituye una proliferación maligna de las células plasmáticas derivadas de un solo clon. El tumor, sus productos y la respuesta del paciente ocasionan diversos trastornos funcionales orgánicos y síntomas como dolores óseos o fracturas, insuficiencia renal, predisposición a infecciones, anemia, hipercalcemia y, en ocasiones, trastornos de la coagulación, síntomas neurológicos y manifestaciones vasculares de hiperviscosidad. Objetivo: Presentar un caso infrecuente teniendo en cuenta que la infección aguda como forma de presentación inicial de mieloma es poco usual. Presentación del caso: Se presenta un caso de un paciente de 61 años de edad, ingresado en el servicio de ortopedia en tres ocasiones diferentes y operado de artritis séptica de la rodilla izquierda en estas tres oportunidades. Se mantuvieron los elementos inflamatorios locales a nivel de la articulación, fiebre recurrente, anemia y eritrosedimentación acelerada. Conclusiones: Los estudios hematológicos específicos confirmaron el diagnóstico de mieloma múltiple. Se comenzó tratamiento de inducción a la remisión para esta enfermedad con melfalán y prednisona; se logró mejoría del cuadro infeccioso al concluir el primer ciclo de esta intervención terapéutica(AU)

ABSTRACT Introduction: Multiple myeloma constitutes a malignant proliferation of plasma cells derived from a single clone. The tumor, its products, and the patient's response cause various organic functional disorders and symptoms such as bone pain or fractures, kidney failure, predisposition to infections, anemia, hypercalcemia, and sometimes coagulation disorders, neurological symptoms, and vascular manifestations of hyperviscosity. Objective: To present a rare case, taking into account that acute infection as the initial presentation of myeloma is unusual. Case report: We report a case of a 61-year-old patient, admitted to the orthopedic service on three different occasions and operated on for septic arthritis of the left knee, in every occasions. The local inflammatory elements at the joint level, recurrent fever, anemia and accelerated erythrocyte sedimentation were still present. Conclusions: Specific hematological studies confirmed the diagnosis of multiple myeloma. Induction-to-remission treatment for this disease was started with melphalan and prednisone; improvement of the infection was achieved at the conclusion of the first cycle of this therapeutic intervention(AU)

Trasplante de progenitores hematopoyéticos autólogo en gammapatías monoclonales: primeros resultados del Hospital "Hermanos Ameijeiras" / Autologous stem cell transplantation in monoclonal gammopathies: first results from the "Hermanos Ameijeiras" Hospital

Introducción: Los avances en el manejo del mieloma múltiple (MM) durante los últimos años incluyen la incorporación del trasplante de progenitores hematopoyéticos autólogo (TPHa) a la estrategia de tratamiento de estos pacientes. Objetivo: Dar a conocer los primeros resultados en el hospital Hermanos Ameijeiras (HHA) con la aplicación del TPHa en pacientes con gammapatías monoclonales (GM), empleando las altas dosis de melfalán (AD-Mel) como tratamiento acondicionante (TA) y su impacto en la sobrevida global (SG). Métodos: Se hizo un estudio retrospectivo de todos los pacientes con GM sometidos a TPHa en el Servicio de Hematología del HHA en el período comprendido entre 2009 y 2018. La muestra final comprendió 14 casos. Resultados: La edad promedio fue de 53,5 años; la mayoría tenía como diagnóstico MM (85,7 por ciento) y todos ellos debutaron en estadio III de Durie-Salmon; como TA el 64,2 por ciento recibió AD-mel, en dosis de 200 mg/m2. La recuperación de las cifras de neutrófilos y plaquetas ocurrieron como promedio a los 11,4 y 12 días, respectivamente. La mortalidad relacionada con el trasplante (MRT) al día +30 fue del 7,1 por ciento. La probabilidad de SG a los 2 años fue superior al 90 por ciento y a los 5 años del 68 por ciento. Conclusiones: Se comprobó que la realización del TPHa con el empleo de AD-Mel como TA en pacientes con GM es un proceder realizable en nuestro país con una MRT relativamente baja. Se logró demostrar que la inclusión del TPH en el tratamiento mejora considerablemente las expectativas de sobrevida de estos pacientes(AU)

Introduction: The recent advances in the management of multiple myeloma (MM) during the last years have included the autologous hematopoietic stem cell transplantation (auto-HSCT) to the treatment strategy of these patients. Objective: To present the first results in the Hermanos Ameijeiras hospital (HAH) with the application of auto-HSCT in patients with monoclonal gammopathies (MG) using high doses of melphalan (HD-Mel) as conditioning regimen (CR) and its impacton overall survival (OS). Methods: A retrospective study of all patients with MG who underwent auto-HSCT in the Hematology Service of the HAH in the period between 2009 and 2018 wasmade. The final sample comprised 14 cases. Results: The average age was 53.5 years; the majority had diagnosis of MM (85.7percent) and all of them were diagnosed in stage III of Durie-Salmon; as CR 64.2 percent received HD-mel, at 200 mg/m2. The recovery of neutrophil and platelet counts occurred on average at 11.4 and 12 days respectively. Transplant related mortality (TRM) at day +30 was 7.1 percent. The probability of OS at 2 years was higher than 90 percent and at 5 years of 68 percent. Conclusions: It was verified that the performance of auto-HSCT with the use of HD-Mel as CR in patients with MG is a feasible procedure in our country with a relatively low TRM. It was possible to demonstrate that the inclusion of auto-HSCT in the treatment considerably improves the survival expectations of these patients(AU)

Impacto de crioterapia como prevención de mucositis / Impact of cryotherapy in preventing mucositis

El mieloma múltiple es la segunda neoplasia hematológica en orden de frecuencia. Se define por la presencia de células plasmáticas monoclonales con capacidad para producir una paraproteína monoclonal y causar alteraciones clínicas en forma de anemia es una patología tratable pero invasiva como consecuencia del tratamiento quimioterápeutico el revestimiento mucoso de la boca sufre alteraciones, lo que puede dar lugar a su inflamación y ulceración, la gravedad de la mucositis oral puede evaluarse utilizando diferentes escalas. Según el grado que alcance puede interferir en el estado funcional y calidad de vida del paciente causando dolor, desnutrición e infecciones locales o sistémicas. Genera retraso del tratamiento, prolonga la estancia hospitalaria, incrementa el gasto sanitario. Una de las medidas preventivas es la crioterapia, que realiza el principio básico de vasoconstricción para limitar la toxicidad local de ciertas terapias, la hipotermia resultante produce una reducción del flujo sanguíneo por efecto de la vasoconstricción con la disminución de los efectos citotóxicos por concentración de los fármacos en el área que se enfrió. La misma está indicada para la prevención de mucositis en drogas de vida media corta, tales como el melfalán en altas dosis.Uno de los objetivos propuestos fue comparar el grado de mucositis alcanzado por los pacientes que realizaron crioterapia de enero a septiembre del año 2017 con lo que no realizaron crioterapia durante el mismo periodo del 2016.

Multiple myeloma is the second most frequent hematologic neoplasia. It is defined by the presence of monoclonal plasmatic cells that can produce a monoclonal paraprotein and cause clinical alterations, like anemia. The pathology is treatable but invasive. The chemotherapy can alter the mucosa lining of the mouth, causing inflammation and ulceration. The seriousness of the oral mucositis can be evaluated by using different scales. Depending on the degree reached, it can interfere in the functional status and life quality of the patient, causing pain, malnutrition and local or systemic infections. It delays the treatment, prolongs the hospitalization and increases the health care costs. One of the preventive steps is cryotherapy that applies the basic principle of vasoconstriction to limit the local toxicity of certain therapies. The resulting hypothermia reduces the blood flow, and diminishes the cytotoxic effects by concentrating the drugs in the area that was chilled. Cryotherapy is indicated to prevent mucositis when short half-life drugs, such as melphalan in high doses, are used. One of the proposed goals was to compare the degree of mucositis developed by patients who received cryotherapy from January to September 2017, to those who did not receive cryotherapy during the same period in 2016.

Trasplante de médula ósea alogénico en un paciente con fusariosis diseminada y leucemia mieloide aguda / Allogeneic hematopoietic cell transplantation in a patient with disseminated fusariosis and acute myeloid leukemia

La infección diseminada por Fusarium se ha convertido en un problema creciente en las personas con neoplasias hematológicas malignas, principalmente en pacientes con leucemias agudas; se describen cada vez más casos en aquellos sometidos a un trasplante de médula ósea. No existe un tratamiento óptimo establecido para la fusariosis diseminada. La mortalidad global comunicada de esta infección oscila entre el 50 y el 80%. Se presenta a continuación el caso de un paciente de sexo masculino de 29 años, con diagnóstico de leucemia mieloide aguda, que presenta como complicación una fusariosis diseminada, y logra sobrellevar un trasplante alogénico de médula ósea en el Hospital Italiano de San Justo (Argentina) de forma exitosa. (AU)

Disseminated fusariosis has become an increasing problem in people with hematopoietic neoplasms, mainly in patients affected by acute leukemias, and even more in those who undergo hematopoietic cell transplantation. There is not an optimal treatment for disseminated fusariosis. The global mortality described in the literature is between 50% and 80%. We introduce a case of a 29 year old patient with diagnosis of acute myeloid leukemia complicated with disseminated fusariosis, who copes with an allogeneic hematopoietic cell transplantation with a successful outcome in the "Hospital Italiano de San Justo" (Argentina). (AU)

Plasmocitoma extramedular: una rara causa de masa mediastínica / Extramedullary mediastinal plasmacytoma: report of one case

Solitary extramedullary plasmacytomas represent 3% of plasma cell neoplasms. Their most common locations are the upper gastrointestinal and respiratory tract. We report a 70-year-old male presenting with progressive dyspnea and cough. A chest radiography showed widened mediastinum. Chest computed tomography showed a mediastinal mass. A bronchoscopy showed an extrinsic compression and complete occlusion of the primary bronchus. A self-expandable prosthesis was installed in left bronchus. A population of plasmacytoid cells was evidenced in the mass biopsy. Immunohistochemistry revealed CD20+ and CD138+. These tumor cells stained positively for lambda light chains, but negatively for kappa chains. Therefore an extramedullary plasmacytoma was diagnosed. A multiple myeloma was discarded with a normal blood count, serum calcium and creatinine levels. Serum protein electrophoresis had a monoclonal spike, serum IgG was 1963 mg/dl. Bone marrow aspiration had 1% of plasma cells. Bone x-rays were normal. Computed tomographies of the neck, thorax, abdomen and pelvis ruled out other plasmacytomas. Eight cycles of chemotherapy with thalidomide, melphalan and prednisone were indicated, obtaining complete remission of the tumor. The review of the literature shows that mediastinal extramedullary plasmacytomas are extremely rare. They generally appear in men aged between 50 and 60 years. The treatment of choice is radiotherapy, but given the location in the present case, chemotherapy was considered the best option. Recurrence is approximately 10 to 30% and 10% of patients progress to myeloma, thus requiring close monitoring.

Clinical assessment of 252Californium neutron intracavitary brachytherapy using a two-channel Y applicator combined with external beam radiotherapy for endometrial cancer

OBJECTIVE: The aim of this study was to determine the efficacy of 252Californium neutron intracavitary brachytherapy using a two-channel Y applicator combined with external beam radiotherapy for the treatment of endometrial cancer. METHODS: Thirty-one patients with stage I-III endometrial cancer were recruited for this study. The stage I patients received only 252Californium neutron intracavitary brachytherapy with a two-channel applicator. The stage II and III patients received both 252Californium neutron intracavitary brachytherapy using a two-channel applicator and parallel-opposed whole pelvic radiotherapy. RESULTS: The five-year local control rate was 80.6% (25/31), the overall survival rate was 51.6% (16/31), and the disease-free survival rate was 54.8% (17/31). The incidence of serious late complications was 12.9% (4/31). CONCLUSIONS: 252Californium neutron intracavitary brachytherapy using a two-channel applicator combined with external beam radiotherapy was effective for treating endometrial cancer and the incidence of serious late complications related to this combination was within an acceptable range.

Sospecha diagnóstica de mieloma múltiple en atención primaria: reporte de un caso / Multiple myeloma suspected in primary care: case report

Introducción: El mieloma múltiple (MM) es una neoplasia hematológica caracterizada por una proliferación clonal de células plasmáticas que genera una disfunción multiorgánica, con daño a nivel óseo, siendo una importante causa de morbimortalidad en estos pacientes. Presentación del caso: Mujer de 69 años, hipertensa tratándose con enalapril hace 10 años, operada de un prolapso genital en el año 2015, con antecedentes de fallecimiento de un hijo a los 30 años por un cáncer no especificado, refirió cuadro de un año de evolución caracterizado por oligoartralgias en extremidades inferiores, con predominio derecho, además de baja de peso de aproximadamente 5 kg en 4 meses. En atención primaria se solicitó exámenes que revelaron anemia moderada normocítica-normocrómica y velocidad de eritrosedimentación de 125 mm/hora, por lo cual, se derivó a Hospital de Puerto Montt. Durante hospitalización, se realizó biopsia de médula ósea que informó células plasmáticas que representaban más del 90% de los elementos medulares evaluables. Electroforesis de proteínas en sangre destacó banda de aspecto monoclonal en zona gamma de 7.9 g/dl. Frotis de eritrocitos con rouleaux positivo, proteínas totales de 13.1 g/dl y calcio corregido de 10.6 mg/dl. Se confirmó MM, iniciándose tratamiento con talidomida, melfalán y prednisona, además de bifosfonato y analgesia, con controles mensuales en policlínico de Hematología. Discusión: El MM es una enfermedad incurable, por ello el diagnóstico precoz en atención primaria es fundamental para dirigir los esfuerzos terapéuticos hacia la remisión de la enfermedad y mejoría de la calidad de vida.

ntroduction: Multiple myeloma (MM) is a hematological neoplasm characterized by clonal proliferation of plasma cells that generates multiple organ dysfunction, with bone damage, which it's an important cause of mortality in these patients. Case report: F69 year old woman with hypertension treated with enalapril from 10 years ago, with history of a genital prolapse surgery in 2015, and refers the death of a 30 years old son for an unspecified cancer. She had a 1 year evolution of symptoms characterized by oligoartralgias in lower extremities, predominantly right, in addition to 5 kg weight loss in 4 months. In Primary Care tests were requested, showing: moderate anemia normocytic- normocromic, erythrocyte sedimentation rate 125 mm/hour. Then, she was referred to Hospital of Puerto Montt to complete the study. Hospitalized, bone marrow biopsy was performed and showed more tan 90% of plasma cells. Blood protein electrophoresis, showed monoclonal band in gamma zone of 7.9 g/dl. Smears erythrocyte rouleaux positive, measurement of total proteins of 13.1 g/dl, corrected calcium of 10.6 mg/dl. MM suspicion was confirmed, starting treatment with thali-domide, melphalan and prednisone plus bisphosphonate and analgesia, with monthly checks of hematology polyclinic. Discussion: The MM is an incurable disease, so early diagnosis in Primary Care is essential to direct therapeutic efforts toward disease remission and improved quality of life.

Isolated limb perfusion with hyperthermia and chemotherapy: predictive factors for regional toxicity

OBJECTIVE: Isolated limb perfusion combined with melphalan is an accepted treatment for obtaining locoregional control in advanced melanoma of the extremities and other malignant neoplasias restricted to the limb. This study aims to examine the factors associated with toxicity caused by the regional method. We considered the technical aspects of severe complications associated with the procedure in an attempt to diminish the patient morbidity that occurs during the learning curve. METHODS: We conducted a retrospective analysis of the records of patients who underwent perfusion at the AC Camargo Hospital in São Paulo, Brazil between January 2000 and January 2009. The Wieberdink scale was applied to classify local toxicity and its relation to clinical and laboratory variables. RESULTS: Fifty-eight perfusions were performed in 55 patients. Most patients (86.2%) presented a toxicity level between I and III. Grade V toxicity was seen in five cases (8.6%), four of which occurred in the first 2 years. Creatine phosphokinase, an important predictive factor for toxicity, had an average value of 231.8 for toxicity grades I-III and 1286.2 for toxicity grades IV-V (p = 0.001). There was a relationship between the melphalan dose and toxicity, which was 77 mg (25 to 130 mg) for toxicity grades I-II and 93.5 mg (45 to 120 mg) for toxicity grades IV-V (p = 0.0204). CONCLUSION: It is possible to prevent the toxicity associated with melphalan by adjusting the dose according to the patient's body weight (especially for women and obese patients) and the creatine phosphokinase values in the postoperative period.

Busulfan and melphalan as conditioning regimen for allogeneic hematopoietic stem cell transplantation in acute myeloid leukemia in first complete remission

BACKGROUND: Allogeneic hematopoietic stem cell transplantation with HLA-identical donors has been established for the treatment of acute myeloid leukemia patients for over 30 years with a cure rate of 50 percent to 60 percent. OBJECTIVES: To analyze the overall survival of patients and identify factors that influence the outcomes of this type of transplant in patients in 1st complete remission who received a busulfan and melphalan combination as conditioning regimen. METHODS: Twenty-five consecutive patients with acute myeloid leukemia were enrolled between 2003 and 2008. The median age was 34 years old (Range: 16 - 57 years). All patients received cyclosporine and methotrexate for prophylaxis against graft-versus-host disease. Median neutrophil engraftment time was 16 days (Range: 7 - 22 days) and 17 days (Range: 7 - 46 days) for platelets. Sinusoidal obstructive syndrome was observed in three patients, seven had grade II acute graft-versus-host disease and one extensive chronic graft-versus-host disease. RESULTS: The overall survival by the Kaplan-Meier method was 48 percent after 36 months with a plateau at 36 months after transplantation. Intensive consolidation with high-dose arabinoside resulted in an improved survival (p-value = 0.0001), as did grade II acute graft-versus-host disease (p-value = 0.0377) and mild chronic graft-versus-host disease (p-value < 0.0001). Thirteen patients died, five due to infection within 100 days of transplant, two due to hemorrhages, one to infection and graftversus-host disease and three relapses followed by renal failure (one) and infection (two). The cause of death could not be determined for two patients. CONCLUSION: The busulfan and melphalan conditioning regimen is as good as other conditioning regimens providing an excellent survival rate.

Condicionamento reduzido no mieloma múltiplo / Reduced conditioning fo multiple myeloma

Os autores descrevem o estudo desenvolvido pelo Grupo Italiano de Transplante de Medula Óssea (GITMO), em 12 centros italianos, entre 2001 e 2005, com a utilização de transplante alogênico com condicionamento de intensidade reduzida (RIC) em mieloma múltiplo. Foram estudados 53 pacientes, a maioria com doença avançada. Os resultados mostraram que o RIC foi factível e com resultados que demonstram que o transplante alogênico pode ser melhorado em termos de toxicidade e evolução clínica utilizando novos regimes de condicionamento e novas drogas.

The authors report a study carried out about GITMO (Italian Group for Bone Marrow Transplantation) including 12 Italian Centers from 2001 to 2005 with allogeneic bone marrow transplantation using reduced intensity conditioning regimen (RIC) in patients with multiple myeloma. Fifty-three patients were enrolled, most of them presenting advanced disease. The study showed that RIC is feasible and suggested that RIC may improve outcome in this setting patients.

Manifestações sistêmicas adversas em medicina intensiva após realização de perfusão isolada de membro com melfalan e hipertermia: relato de caso / Adverse systemic reactions in intensive care medicine after isolated limb perfusion with melphalan and hyperthermia: case report

JUSTIFICATIVA E OBJETIVOS: A presença de efeitos adversos, inerentes a todos os tratamentos, justifica a necessidade do profundo conhecimento pela equipe médica para prevenção e tratamento de eventuais disfunções orgânicas, reduzindo o seu impacto. O objetivo deste estudo foi relatar um caso de paciente que apresentou diversas manifestações sistêmicas, após a realização de perfusão isolada de membro com melfalan e hipertermia. RELATO DO CASO: Paciente do sexo feminino, 64 anos, branca, com diagnóstico de melanoma na região de maléolo medial do membro inferior esquerdo. Seis meses após a excisão da lesão, realizou-se perfusão isolada do membro, com melfalan e hipertermia para conter o processo em evolução de possível metástase. A admissão na UTI apresentou síndrome da resposta inflamatória sistêmica (SIRS) com instabilidade hemodinâmica refratária à expansão volêmica. Durante a internação evoluiu com quadro de edema agudo de pulmão e disfunção miocárdica, revertidos com sucesso, depois de adequada intervenção terapêutica. CONCLUSÕES: A presença de efeitos adversos, inerentes a todos os tratamentos oncológicos, justifica a necessidade do conhecimento pela equipe da terapia intensiva para prevenção e tratamento de eventuais disfunções orgânicas, reduzindo o impacto de sua morbimortalidade.

BACKGROUND AND OBJECTIVES: The presence of adverse reactions, inherent to all treatments, justifies the necessity of deep knowledge, by the medical team of the prevention and treatment of occasional organic dysfunctions, reducing its impact. The purpose of this paper is to report a case comprising the several systemic adverse reactions after perfusion of limb with melphalan and hyperthermia. CASE REPORT: A white female, 64-years old patient with diagnosis of melanoma in the medial malleoli region of the left lower limb. Six months after surgical removal of wound, an isolated perfusion of limb was carried out with melphalan and hyperthermia in order to curb the possible metastatic process in evolution. At admission in the ICU, the patient presented systemic inflammatory response syndrome (SIRS) with refractary hemodynamic instability to volemic expansion. During internation the patient evolved to acute lung edema and myocardial dysfunction, all reverted successfully. CONCLUSIONS: The potential presence of adverse reactions, inherent to all treatments, justify the necessity of knowledge by the intensive care team in the prevention and treatment of occasional organic dysfunctions, reducing the impact of morbidity and mortality.

Síndrome de lisis de glóbulos blancos después de un trasplante autólogo de células troncales hematopoyéticas en el tratamiento de la amiloidosis AL renal: Caso clínico / White blood cell lysis syndrome after autologous peripheral blood stem cell transplantation in the treatment of renal AL amyloidosis: Case report

The treatment of AL amyloidosis was not successful until the advent of myeloablative chemotherapy consisting of high-dose intravenous melphalan followed by autologous peripheral blood stem cell transplantation. This new treatment has achieved better survival rates and, remarkably, it has obtained complete remission. Among patients with renal involvement, achievement of a complete hematological response was associated with a 50% reduction in proteinuria and stable creatinine clearance in more than 2/3 of patients. Despite of these excellent results, this new therapy is associated with significant toxicity, including the development of acute renal failure due to white blood cell lysis syndrome. We report a 59 year-old female with a nephrotic syndrome due to primary amyloidosis successfully treated autologous stem cell transplantation who developed acute renal failure caused by white blood cell lysis syndrome. The patient required treatment with granulocytic colony stimulating factor and intermittent hemofiltration and was discharged 23 days after melphalan administration with a satisfactory renal function and white blood cell count. After one year of follow up, she maintains a good glomerular filtration rate, a proteinuria of less than, 1 g/day and normal hematological values.

Histamine combined with melphalan in isolated limb perfusion for the treatment of locally advanced soft tissue sarcomas: preclinical studies in rats

OBJETIVO: Avaliar o potencial benéfico da histamina combinada ao melfalano, na perfusão de membro isolado (PMI), como alternativa à combinacão TNF-alfa mais melfalano, no tratamento de sarcomas de partes moles irressecaveis em extremidades, em ratos de linhagem Brown Norway (BN). MÉTODOS: 20 ratos BN foram submetidos a implantacão de fragmentos de fibrosarcoma singênico BN-175 na pata traseira direita. Em cerca de 7-10 dias o tumor atingiu um diâmetro médio de 12-15 mm e foram aleatóriamente divididos em quatro grupos (controle, melfalano,histamina em doses progessivas combinada ao melfalano e histamina) sendo submetidos a PMI experimental por 30 minutos. Os tumores foram então medidos diariamente com o uso de paquímetro e o volume tumoral calculado. RESULTADOS: As curvas de resposta mostram um efeito significativo da combinacão de Histamina na concentracão de 200 mg/mL ao melfalano, com 66% de resposta global incluindo 33% de respostas completas (p < 0.01). Não houve efeitos colaterais sistêmicos e localmente apenas edema leve e transitório nos animais tratados com histamine. CONCLUSAO: A histamina em combinacão com o melfalano apresenta um efeito promissor na PMI garantindo maiores investigacões do seu mecanismo de acão e do seu potencial uso na perfusão de órgãos.

Trasplante de células hematopoyéticas de sangre periférica utilizando quimioterapia inmunosupresora sin destrucción de la médula ósea: "minitrasplante" / Peripheral blood hematopoietic cell transplant using immunosuppressive chemotherapy without bone marrow destruction: minitransplant

Using a nonmyeloablative, immunosuppressive, fludarabine (FLU)-base conditioning regimen, we have performed allogeneic peripheral blood stem cell transplants in 17 patients (six with chronic granulocytic leukemia, four with acute myelogenous leukemia, five with acute lymphoblastic leukemia, one with myelodysplasia and one, with thalassemia major). Conditioning regimen consisted of FLU/busulfan/cyclophosphamide or FLU melphalan. To avoid graft vs. host disease (GVHD), cyclosporine and methotrexate were used. Median granulocyte recovery time to 0.5 x 10(9) was 11 days, whereas median platelet recovery time to 20 x 10(9) was 12 days. Seven patients did not need red blood cell transfusions and four did not need platelet transfusions. In thirteen individuals (76), the procedure could be completed fully on an outpatient basis. Follow-up times range between 1 and 14 months. Five of 17 patients developed acute GVHD whereas 4/10 developed chronic GVHD. The 14-month survival (SV) is 70 and median SV is not reached. Five patients (29) have died, three due to relapse of the disease and two due to GVHD. The transplant-related mortality was 5.8. This procedure is substantially less costly than its counterpart, using in-hospital myeloablative conditioning regimens, and may represent another approach in management of patients requiring allogeneic stem cell transplant.

Mieloma múltiplo: relato de caso / Multiple myeloma: case report

Os autores apresentam um caso clínico de um paciente portador de mieloma múltiplo, com anemia, proteinúria, dores ósseas, velocidade de hemossedimentação elevada, em que faltou a positividade da proteinúria de Bence Jones, e a eletroforese das proteínas plasmáticas não apresentava o pico monocional da doença

Mieloma múltiplo com pico monoclonal na fraçäo beta / Multiple myeloma with a monoclonal peak at beta-globulin fraction: case report

É relatado o caso de um paciente idoso, com quadro agudo de dorsalgia e dasabamento da sétima vértebra torácica, cuja eletroforese de proteínas séricas demonstrava um pico monoclonal na fraçäo beta-globulinas, achado pouco freqüente, encontrado em apenas 10 por cento dos casos de mieloma múltiplo. O paciente foi submetido a uma punçäo aspirativa de medula óssea via esternal. No mielograma observou-se 70 por cento de plasmócitos isolados e agregados, alguns displásicos e binucleados, compatível com mieloma múltiplo. Iniciou-se quimioterapia em janeiro de 2000, com Alkeran e Prednisona, tendo o paciente rapidamente apresentado melhora clínico-laboratorial.

Macroglobulinemia de Waldenstron / Waldestrom's macroglobulinemia

Se presenta el caso clínico de un hombre de 70 años que consultó luego de varios meses de mareos, cansancio muscular, baja de peso y palidez. El estudio realizado demostró: pancitopenia con anemia normocítica, normocrómica, arregenerativa; hiperproteínemia con un componente monoclonal; gammapatía monoclonal IgM kappa con cadena liviana lambda libre; ausencia de lesiones osteolíticas; médula ósea con infiltración linfomatosa y disminución de las series eritroide, mieloide y megacariocítica pero sólo con 3 por ciento de plasmocitos; hiperviscosidad sanguínea. Luego de dos ciclos de melfalan y prednisona el paciente reingresa con un síndrome febril y compromiso de conciencia y fallece a los pocas horas con una sepsis a gram negativos (Escherichia coli) y una infección meníngea