Synchronous diagnosis of anaplastic large cell lymphoma and multiple myeloma in a patient: A case report.

RATIONALE: Synchronous development of both anaplastic large cell lymphoma (ALCL) and multiple myeloma (MM) in a patient is rare. To our knowledge, until now only one case has been reported. Treatment needs to cover both and is a challenge. Here we reported another case and discussed the diagnosis and treatment. PATIENT CONCERNS: This is a 63-year old woman who presented with a mass in upper abdominal skin. Positron emission tomography/computed tomography (PET/CT) showed the high metabolism in left abdominal skin and left axillary lymph nodes. Histopathologic and immunohistochemical evaluation identified the cutaneous mass as an ALK-negative ALCL. Bone marrow smear showed increased plasma cells which expressed CD38, CD138, and cLambda concomitantly. The increased monoclonal immunoglobulin IgD λ was detected by immunofixation electrophoresis. DIAGNOSES: Diagnosis of both ALCL and MM was confirmed. INTERVENTIONS: The patient successively received 6 cycles of B-CHOD regimen, one cycle of ID regimen, 2 cycles of DHAX regimen, one cycle of L-DA-EPOCH and autologous stem cell transplantation (ASCT). Then lenalidomide was performed as a maintenance therapy. OUTCOMES: Both ALCL and MM achieved complete remission. LESSONS: We reported a very rare case with synchronous development of ALCL and MM, in whom a good therapeutic response to chemotherapies followed by ASCT has been observed.

Mieloma múltiple en paciente joven / Multiple Myeloma in Young Patient

Introducción: el mieloma múltiple es una enfermedad hematológica maligna caracterizada por una proliferación de células plasmáticas en la médula ósea. La edad más común del inicio es entre 65 y 70 años, sin embargo se documentan casos en jóvenes en formas cada vez más graves. Presentación de caso: hombre de 31 años, que cursa con dolores óseos generalizados, pérdida de peso y adinamia, de 6 meses de evolución. Se constatan cifras de creatinina y calcio sérico elevadas durante su hospitalización, presenta proteína de Bence-Jones positiva para cadenas ligeras Kappa, y se realiza biopsia de médula que concluye en mieloma plasmoblástico. Conclusiones: se comienza tratamiento con citostático asociado a esteroides. La presentación antes de los 40 años es infrecuente y el pronóstico, sombrío, a pesar del tratamiento oncoespecífico(AU)

Introduction: Multiple myeloma is a malignant hematologic disease characterized by a proliferation of plasma cells in the bone marrow. The most common age of onset is between 65 and 70 years, however cases are documented in young people in increasingly severe forms. Case presentation: A case of a 31-year-old man, with generalized bone pain, weight loss and adynamia, of 6 months of evolution is presented here. Serum creatinine and serum calcium levels are high during hospitalization, Bence-Jones protein is positive for Kappa light chains, and marrow biopsy is performed, which concludes in plasmoblastic myeloma. Conclusions: Treatment with cytostatic associated with steroids is started. This onset before age 40 is not frequent, and prognosis is bleak, despite the specific oncology treatment(AU)

Changing patterns of AIDS: impact on the indications and diagnostic yield of bone marrow biopsies.

After the advent of HAART, the clinical course of HIV infection has dramatically improved. Therefore, it seems appropriate to reevaluate the performance of bone marrow biopsy (BMB) as a diagnostic tool. The aim of the present study was to compare the reasons for performing a BMB and its diagnostic yield in HIV-patients before and after HAART. A total of 165 BMB specimens obtained from HIV-infected patients receiving care at the Hospital of Universidade Federal do Rio de Janeiro in two different periods (1986-1994 and 1999-2004) were analysed. The main reason for BMB examination in the first period was fever (88%), which decreased in the second period (57%, p < 0.0001), when cytopenia (51%) was the leading reason for BMB, whereas in the first period it accounted for only 30% (p = 0.008). A definitive diagnosis (infection, granulomas or lymphomas) was obtained in 28% of patients in the first period and in 19% during the second period (p = 0.20). The diagnosis turned out as infections decreased from 16% in period 1 to 2% in period 2 (p = 0.003). Despite the the limitations in the evaluation of fever, the use of BMB must be considered on an individual basis, whenever less invasive alternatives have been exhausted, and should be complemented by a bone marrow aspiration for microbiological studies.

Changing patterns of AIDS: impact on the indications and diagnostic yield of bone marrow biopsies

After the advent of HAART, the clinical course of HIV infection has dramatically improved. Therefore, it seems appropriate to reevaluate the performance of bone marrow biopsy (BMB) as a diagnostic tool. The aim of the present study was to compare the reasons for performing a BMB and its diagnostic yield in HIV-patients before and after HAART. A total of 165 BMB specimens obtained from HIV-infected patients receiving care at the Hospital of Universidade Federal do Rio de Janeiro in two different periods (1986-1994 and 1999-2004) were analysed. The main reason for BMB examination in the first period was fever (88 percent), which decreased in the second period (57 percent, p < 0.0001), when cytopenia (51 percent) was the leading reason for BMB, whereas in the first period it accounted for only 30 percent (p = 0.008). A definitive diagnosis (infection, granulomas or lymphomas) was obtained in 28 percent of patients in the first period and in 19 percent during the second period (p = 0.20). The diagnosis turned out as infections decreased from 16 percent in period 1 to 2 percent in period 2 (p = 0.003). Despite the the limitations in the evaluation of fever, the use of BMB must be considered on an individual basis, whenever less invasive alternatives have been exhausted, and should be complemented by a bone marrow aspiration for microbiological studies.

Safety of general anesthesia for lumbar puncture and bone marrow aspirate/biopsy in pediatric oncology patients.

INTRODUCTION: Painful short duration procedures such as bone marrow aspiration/biopsy and the lumbar puncture with or without intrathecal chemotherapy are frequently performed during the treatment of children with cancer. This study evaluated the frequency and severity of complications of bone marrow aspiration biopsy and lumbar puncture/intrathecal chemotherapy under general anesthesia. PATIENTS AND METHODS: A prospective observational study was performed from November 2003 to August 2005. Patients with cancer younger than 21 years old, receiving treatment at the Pediatric Oncology Unity of Hospital de Clínicas de Porto Alegre, undergoing diagnostic and/or therapeutic short duration procedures carried out under general anesthesia in the outpatient surgery unit. RESULTS: One hundred and thirty-seven patients were submitted to 423 procedures under general anesthesia. There were 61% boys, mean age of 7.5 years (0.2 to 21) and ASA II 98%. Eighty seven percent of the procedures were carried out in patients with leukemia or lymphoma. The majority of the procedures had no adverse events during intraoperative and postoperative periods. No procedure had to be suspended after it had begun. One patient had lumbar pain after the procedure and was admitted to the ward with suspected subdural bleeding, but this was not confirmed. No patient needed cardiopulmonary reanimation or treatment in the intensive care unit. CONCLUSIONS: General anesthesia for short duration painful procedures in children undergoing treatment for malignancies is safe when carried out by trained professionals in outpatient clinical surgery unit.

É necessária a realização de biópsia de medula óssea bilateral para o estadiamento do linfoma difuso de grandes células B? / Is it necessary to perform a bilateral bone marrow biopsy to stage diffuse large B-cell lymphoma?

Estudo retrospectivo que visa analisar a utilidade da biópsia de medula óssea (BMO) bilateral na infiltração de medula óssea (MO) por linfoma difuso de grandes células B (LDGCB). Nossos objetivos foram avaliar a incidência de infiltração unilateral de MO por LDGCB e comparar o comprimento dos fragmentos obtidos entre as amostras positivas e negativas para infiltração. Além disso, verificamos se houve diferença entre os casos com infiltração unilateral versus bilateral, correlacionando com desidrogenase láctica (DHL) e estadiamento tomográfico. Avaliamos 268 casos de LDGCB e observamos infiltração medular em 34 casos (13 por cento). Não foi possível a avaliação de seis casos, restando 28 casos para análise. Foram revisados no total 70 fragmentos de MO sobre presença ou ausência de infiltração e comprimento. A média do número de fragmentos por casos foi 2,5; a média do comprimento dos fragmentos foi 11,01 mm (± 5,12 mm), e a média do comprimento dos fragmentos por caso foi 27,53 mm. Foi observado que em seis casos (21,4 por cento) havia infiltração unilateral. Não foram evidenciadas diferenças nas médias do comprimento dos fragmentos em relação à presença versus ausência de infiltração 10,95 mm (± 5,1 mm) versus 11,57 mm (± 5,2 mm), p > 0,05, respectivamente. Não foram evidenciadas diferenças em 23 casos entre a comparação da infiltração medular unilateral versus bilateral com DHL e estadiamento tomográfico. Concluímos que a BMO bilateral foi superior à unilateral, pois pode aumentar a detecção de infiltração de MO em 21,4 por cento dos casos.

This retrospective study aims to analyze the usefulness of bilateral bone marrow biopsy in bone marrow infiltration by diffuse large B-cell lymphoma (DLBCL). Our objectives were to assess the incidence of unilateral BM involvement by DLBCL and compare fragment length obtained from positive and negative samples for infiltration. Furthermore, we compared the differences between unilateral and bilateral infiltration correlating with lactic dehydrogenase (LDH) and computerized tomography (CT) staging. We evaluated 268 cases of DLBCL and observed medullary infiltration in 34 cases (13 percent). It was not possible to evaluate 6 out of 34 cases. 70 BM fragments were reviewed as to the presence or absence of infiltration and length. The mean number of fragments per case was 2.5; the mean BM fragment length was 11.01 mm (± 5.12 mm) and the mean BM fragment length per case was 27.53 mm. There was unilateral BM infiltration in six cases (21.4 percent). There were no differences in the mean fragment length as to the presence/absence of infiltration 10.95 mm (± 5.2 mm) versus 11.57 mm, p > 0.05, respectively. There were no differences in 23 cases between the comparison of unilateral medullary infiltration versus bilateral with lactic dehydrogenase and CT staging. We concluded that bilateral bone marrow biopsy was superior to unilateral because it may increase by 21.4 percent the detection of BM involvement by DLBCL.

Compreendendo a experiência de crianças e adolescentes submetidos à punção de medula óssea e lombar em centro cirúrgico de um hospital oncológico / Understanding the experience of children and adolescents to undergo bone marrow aspiration and lumbar surgery center in a hospital cancer

Atualmente, o câncer infantil é bastante compreendido e estudado, apresentando altas probabilidades de cura, graças aos avanços da ciência e da tecnologia. Porém, quando este diagnóstico é dado a crianças ou adolescentes ele ainda gera desconfortos, devido ao impacto que esta doença pode causar no desenvolvimento destes indivíduos. Tanto a fase de diagnóstico, quanto as etapas do tratamento incluem a realização de procedimentos invasivos, bem como a utilização de drogas agressivas. O objetivo deste estudo foi compreender a experiência de crianças e adolescentes submetidos a procedimentos de punção de medula óssea e punção lombar realizadas em uma Unidade de Centro Cirúrgico de um Hospital Oncológico, sedadas antes da realização dos mesmos. Foi realizado um estudo qualitativo, utilizando-se o Discurso do Sujeito Coletivo (DSC) para a organização dos dados, e para análise dos resultados foi elaborado um Quadro Teórico. Foram entrevistados 15 sujeitos nos períodos pré e pós-procedimentos, na faixa etária de 7 a 14 anos de idade, procedentes de diversos Estados do Brasil. A maioria deles é do sexo masculino, com diagnóstico de Leucemia Linfoblástica Aguda (LLA). De acordo comos resultados foram construídos 18 DSC que revelaram, além de aspectos positivos quanto à importância da sedação antes da execução dos procedimentos, noções sobre o tratamento e a doença, assim como, satisfação pelo atendimento recebido, sentimentos como medo, ansiedade, devido ao tempo de espera na etapa do pré-procedimento e insegurança quanto à cura. Foram reveladas também dificuldades quanto à realização do tratamento, como a dependência de transporte público, da rotina hospitalar, a separação da família, a distância da escola e dos amigos. Oferecer uma assistência tecnicamente diferenciada ainda é insuficiente para minimizar as adversidades enfrentadas por estes indivíduos e suas famílias...

Erythrovirus B19 infection in acquired immunodeficiency syndrome: screening by histopathology, immunohistochemistry, and in situ hybridization.

Erythrovirus B19 infects erythrocytic progenitors, transiently interrupting erythropoiesis. In AIDS patients it causes chronic anemia amenable to treatment. We looked for evidences of B19 infection in stored bone marrow material from patients with acquired immunodeficiency syndrome. Histological sections were made from stored paraffin blocks from 33 autopsies (39 blocks) and 35 biopsies (45 blocks, 30 patients) performed from 1988 to 2002. They were examined after hematoxylin-eosin (HE) staining, immunohistochemical (IHC), and in situ hybridization. HE revealed intra-nuclear inclusion bodies ("lantern cells") suggesting B19 infection in 19 sections corresponding to 19 of 63 patients examined with this test. Seven of 78 sections subjected to immunohistochemistry were positive, corresponding to 7 of 58 patients examined with this test. Fourteen sections corresponding to 13 of the 20 HE and/or IHC positive patients were subjected to in situ hybridization, with six positives results. Among the 13 patients subjected to the three techniques, only one gave unequivocal positive results in all and was considered a true positive. The frequency of B19 infection (1/63 patients) in the material examined can be deemed low.

Erythrovirus B19 infection in acquired immunodeficiency syndrome: screening by histopathology, immunohistochemistry, and in situ hybridization

Erythrovirus B19 infects erythrocytic progenitors, transiently interrupting erythropoiesis. In AIDS patients it causes chronic anemia amenable to treatment. We looked for evidences of B19 infection in stored bone marrow material from patients with acquired immunodeficiency syndrome. Histological sections were made from stored paraffin blocks from 33 autopsies (39 blocks) and 35 biopsies (45 blocks, 30 patients) performed from 1988 to 2002. They were examined after hematoxylin-eosin (HE) staining, immunohistochemical (IHC), and in situ hybridization. HE revealed intra-nuclear inclusion bodies ("lantern cells") suggesting B19 infection in 19 sections corresponding to 19 of 63 patients examined with this test. Seven of 78 sections subjected to immunohistochemistry were positive, corresponding to 7 of 58 patients examined with this test. Fourteen sections corresponding to 13 of the 20 HE and/or IHC positive patients were subjected to in situ hybridization, with six positives results. Among the 13 patients subjected to the three techniques, only one gave unequivocal positive results in all and was considered a true positive. The frequency of B19 infection (1/63 patients) in the material examined can be deemed low.

Segmentación de biopsias de médula ósea mediante filtros morfológicos y rotulación de regiones homogéneas / Segmentation of bone marrow biopsies by morphological filters and labeling of flat zones

Este trabajo presenta un método semi-automático de detección de tejidos em biopsias de médula óssea utilizando técnicas de Processamento Digital de Imagens. Las técnicas utilizadas combinan filtrados morfológicos y detección de regiones homogéneas, con el fin de realizar un cálculo preciso de la celularidad medular. Os informes anátomo-patológicos de estos cortes histológicos entregan resultados porcentuales de la celularidas medular, indicando la prsencia de trabéculas, células adiposas y hamatopoyéticas. Dichos porcentajes permiten evalular la presencia y/o el grado de algún desorden metabólico, estableciendo comparaciones entre los valores normales y los patológicos. Generalmente, estas mediciones se realizam por simples inspección visual. El método propuesto permite calcular el porcentaje de trabéculas, células adiposas y hematopoyéticas. La identificación de trabéculas se basa en la aplicación de Filtros Morfológicos Alternativos Secuenciales por Reconstrucción y rotulación de regiones homogéneas. Todo el proceso es implementado en lenguaje MatLab® 6.5 con una interfase visual que permite operar el sistema sin ser experto en Procesamiento de Imágenes. El método fue probado con 26 imágines de biopsias de médula óssea que presentam distribuciones variadas de trabéculas. Se presenta un análisis comparativo entre los resultados obtenidos con el método propuesto y los calculados por dos patólogos especialistas donde la diferencia entre ambas estimaciones es menor al 12 por cento, mientras que las diferencias entre ellos llegan a ser has de 20 por cento. Tanto los resultados visuales como los numéricos indican que el método es altamente eficiente para esta aplicación.

Concomitância de leishmanioses e infecçäo pelo vírus da imunodeficiência humana (HIV): estudo de quatro casos / Concurrent leishmaniasis and human immunodeficiency virus infection: a study of four cases

Säo apresentados quatro casos de leishmaniose em pacientes com SIDA, sendo dois de leishmaniose visceral e dois com forma cutâneo-mucosa. Lesöes cutâneas e de mucosa oral, disseminadas, estavam presentes nos pacientes com a forma tegumentar da doença. Febre prolongada, hepatoesplenomegalia e pancitopenia foram as manifestaçöes principais da forma visceral. A contagem de linfócitos TCD4+ era baixa em todos os casos. A pesquisa de leishmanias foi positiva no aspirado de medula óssea e na biópsia de lesöes cutâneas e mucosas. Os pacientes responderam ao tratamento com antimoniais pentavalentes e com a anfotericina B. Poucos casos de coinfecçäo leishmaniose e HIV têm sido descritos em nosso meio. Apesar da ampla expansäo de ambas, até agora suas áreas de distribuiçäo geográfica teve pouca superposiçäo. Os autores recomendam a inclusäo desta parasitose no diagnóstico diferencial das doenças oportunistas que acometem indivíduos com SIDA

[Diagnosis of blackleg by direct immunofluorescence in bovine bone marrow]. / Diagnóstico de la mancha por inmunofluorescencia directa en médula ósea de bovino.

On smears of marrow-bone death bovines, presumptively diagnostic of black-leg, we have carried out the direct immunofluorescence test (IF). We used two labelled immunosera with fluorescein isothiocyanate. The immunosera wer prepared with the reference strain 5078 of Clostridium chauvoei as following: a) a cellular extract obtained with veronal buffer 0.045 M pH = 8.6, and b) a flagellar suspension obtained by agitation with glass beads, centrifuged at 3,500 x g, and centrifuged again at 16,000 x g during 20 min at 4 degrees C. Both antigenic preparations were injected into rabbits, five doses (0.2, 0.4, 0.8, 1.6 and 3.2 ml) each by i.v. route. Control positive strain of C. chauvoei were used, and control negative strains of C. septicum and C. perfringens were used too. Of all 56 examined samples, 26 (47.5%) gave positive IF test. These results had a 100% of correlation by culture and biochemical identification.

A quantitative method for evaluating the effects of drugs on bone marrow cellularity using the same experimental animal. Quantification of the effects of a single dose of busulphan.

A method for quantifying the cellularity of rats bone marrow per unit of weight is described. Absolute numbers of each cell type per mg of bone marrow in the left and right femurs of the same experimental animal were determined at different times. In normal rats in which both femurs were studied simultaneously it was found that the absolute counts of each cell type per mg of bone marrow in the left and right femurs did not differ, nor were differences found in absolute numbers of marrow cells when the quantitative analyses from the left femurs were compared with those of the right femurs of the same animal, 10 and 20 days later. In order to test the validity of the present method for evaluating the effects of drugs on hematopoiesis, a single oral dose of busulphan (20 mg/kg), was administered to normal rats immediately after the marrow quantitative studies of the left femurs were performed. A marked and significant reduction in total nucleated cell was seen in marrows from the right femurs, 10 days later. Cellular effects were particularly pronounced on the myeloid line. Results presented here indicate that the quantitative study employed is a simple and useful method to evaluate the effects of drugs on hematopoiesis. The novelty of this method lies in: 1) its expression of cellularity on a per mg marrow basis, thereby avoiding possible misinterpretations of data which occur when results are expressed as percentages and 2) the analysis of contralateral femoral marrow specimens obtained from the same animal before and after drugs treatment. Therefore, each animal acts as its own control avoiding possible errors in the determination of drug-induced hematopoietic changes due to inter-animal variability.

A quantitative method for evaluating the effects of drugs on bone marrow cellularity using the same experimental animal: quantification of the effects of a single dose busulphan

Se describe un método que permite cuantificar la celuradidad de la médula ósea de la rata por unidad de peso. A tal efecto se determinaron en diferentes tiempos los números absolutos de cada tipo celular presentes en el fémur derecho e isquierdo del mismo animal de experimentación y los resultados se expresan en número de células por mg de médula ósea. En la rata normal se demostró que el número absoluto de cada tipo celular por mg de médula ósea es muy similar en el fémur derecho e izquierdo. Esto ocurre cuando el estudio de la celularidad medular se realiza simultáneamente en ambos huesos, y también cuando se comparan los resultados del estudio cuantitativo del fémur izquierdo con los del fémur derecho del mismo animal con 10 y 20 días de intervalo. Con el objeto de ratificar la validez del método para el estudio de la acción de drogas sobre la hematopoyesis medular, se observaron, además, los efectos de una dosis de busulfán (20 mg/Kg, vía oral), administrada inmediatamente después de que se realizó el estudio cuantitativo de la celularidad de la médula ósea del fémur izquierdo de una rata normal. Los resultados se compararon con los obtenidos de la médula ósea del fémur derecho de los mismos animales, 10 días después. Se demostró una marcada y significativa disminución del total de células nucleadas por mg de médula ósea, luego de ese período de observación. Los efectos celulares del busulfán fueron particularmente intensos sobre la progenie mieloide de médula ósea, con una reducción del