Evaluación del tratamiento de linfoma de Hodgkin con esquema ABVD en Chile / Treatment of Hodgkin lymphoma: analysis of 915 patients

Background: Hodgkin lymphoma has a high rate of curability, even in advanced stages. Aim: To assess the results of Hodgkin lymphoma treatment using the ABVD (doxorubicin, bleomycin, vinblastine, dacarbazine) chemotherapy regimen. Material and Methods: Analysis of a database held by the Chilean Ministry of Health, including all patients treated at accredited cancer treatment centers. Results: Data for 915 patients, median age 35 years (range 15-86 years) and followed for a median of 97 months (range 1-347 months) were analyzed. Forty-one percent had localized disease. Overall survival at five years for localized and advanced stages was 92% and 74%, respectively. The figures for progression free survival were 87% and 64%, respectively. Patients with relapse who received autologous stem cell transplantation (ASCT) had a five year overall survival of 92%, compared to 64% among those who did not undergo this procedure (p < 0.01). The Guarantees in Health Program set up by the Ministry of Health, was associated with earlier stage disease at diagnosis. Conclusions: The ABVD regimen achieves high rates of cure in localized stages of the disease but the results in advanced stages are not optimal. ASCT significantly improves survival in patients with relapse. The Guarantees in Health Program is associated with earlier diagnosis of the disease.

Necrolisis epidérmica tóxica en el curso de un síndrome hemofagocítico secundario a linfoma de Hodgkin: caso clínico / Toxic epidermal necrolysis during a hemophagocytic syndrome secondary to Hodgkin lymphoma: report of one case

Toxic epidermal necrolysis (TEN) is a lethal entity, characterized by extensive epidermal necrosis and multiorgan failure. Hemophagocytic syndrome (HFS) is also a rare and lethal syndrome characterized by hyperinflammation that leads to the appearance of fever, pancytopenia, organomegaly and hemophagocytosis. The concomitance of these diseases is extremely uncommon. We report a 38 years old female, who during the course of a HFS secondary to Hodgkin Lymphoma (HL), presented a TEN secondary to antibiotics. She was admitted due to a consumptive syndrome, lymphadenopathy, visceromegaly and severe pancytopenia. Laboratory and bone marrow tests confirmed HFS. Due to constant fever, imipenem was indicated. On the third day she started with pain and skin rash. She evolved with positive Nikolsky sign. Cutaneous biopsy was concordant with extensive TEN, which was managed with intravenous immunoglobulin and dexamethasone. A complete response and normalization of the blood count were achieved. Finally, the lymph node biopsy showed HL of mixed cellularity type, which was managed with 8 cycles of ABVD chemotherapy, achieving complete remission.

Prolonged lymphocytosis as the first manifestation of Hodgkin-like adult T-cell leukemia/lymphoma

Abstract Hodgkin-like ATLL is a rare variant of adult T-cell leukemia/lymphoma (ATLL), a disease caused by human T-cell lymphotropic virus type-1 (HTLV-1). At admission, a 46-year-old female presented with lymphadenomegaly, lymphocytosis, slight elevation of LDH blood level, and acid-alcohol resistant bacilli in sputum and was being treated for pulmonary tuberculosis (Tb). She had lymphocytosis in the previous 20 months. Serology for HTLV-1 was positive. Lymph node was infiltrated by medium-sized lymphocytes with scattered Hodgkin and Reed-Sternberg-like cells CD30+, CS1-4+, and CD79a+. Background cells were CD4+ and CD25+. A clinical diagnosis of favorable chronic ATLL was given. She was treated with chemotherapy but later progressed to acute ATLL and ultimately died. Hodgkin-like ATLL should be considered in the histological differential diagnosis with Hodgkin lymphoma since treatment and prognosis of these diseases are distinct. It is also important to search for HTLV-1 infection in patients with unexplained prolonged lymphocytosis.

Lesiones orales asociadas con la enfermedad del virus de inmunodeficiencia humana en pacientes adultos, una perspectiva clínica / Oral lesions associated with human immunodeficiency virus disease in adult patients, a clinical perspective

Among the clinical manifestations which may occur in HIV/AIDS patients, oral lesions are relevant because there are easily accessible and usually the diagnosis is made through clinical features. Some oral manifestations are strongly related with HIV/AIDS patients indicating infection and progression to AIDS and also allow monitoring the success or failure of using antiretroviral therapy. The massive introduction of antiretroviral therapy has changed the morbidity and mortality, frequency, type of clinical manifestation and the timing of the classic opportunistic complications. The aim of this review is to provide an updated of the classical clinical features of the most frequent and relevant HIV/AIDS oral manifestations, considering the fundamental clinical features for their diagnosis.

Dentro de las manifestaciones que pueden aparecer en los pacientes con infección por VIH/SIDA, las lesiones de la cavidad oral tienen gran relevancia debido a que son fácilmente accesibles y por lo general su diagnóstico se efectúa a través de signos clínicos. Además, algunas manifestaciones orales están fuertemente relacionadas con el síndrome por lo que indican sospecha de infección y progresión a etapa SIDA y permiten monitorizar el éxito o fracaso de la terapia anti-retroviral empleada. La introducción masiva de la terapia anti-retroviral ha modificado la morbi-mortalidad, la frecuencia, el tipo de manifestación clínica y el momento de aparición de las clásicas complicaciones oportunistas. El objetivo de esta revisión es entregar las características clínicas clásicas actualizadas de aquellas manifestaciones orales asociadas a la infección por VIH/SIDA que son más frecuentes y que tienen mayor relevancia clínica, considerando las características fundamentales para su diagnóstico.

Validation of the Comfort scale for relatives of people in critical states of health / Validação da escala de conforto para familiares de pessoas em estado crítico de saúde / Validación de la escala de confort para familiares de personas en estado crítico de salud

AbstractObjective: this methodological study aims to present the construct validity of the Comfort scale for family members of people in a critical state of health (ECONF).Method:this is a methodological study. The sample was made up of 274 family members of adults receiving inpatient treatment in six Intensive Care Units (ICU) in the State of Bahía responded to 62 items distributed in 7 dimensions. The validation procedures adopted were based on the techniques of the Classical Test Theory.Results: the analysis of dimensionality was undertaken through principal components analysis, a scale being obtained with 55 items distributed in four factors: Safety, Support, Family member-relative interaction and Integration with oneself and the everyday. The analysis of the items' , discriminative power, undertaken by the item-total correlation-coefficient showed a good relationship of the items with their respective factors. From the ECONF's reliability test, from the analysis of internal consistency, a raised Alpha Cronbach coefficient was obtained for the 4 factors and the general measurement.Conclusion:the comfort scale presented satisfactory psychometric parameters, thus constituting the first valid instrument for evaluating the comfort of family members of people in a critical state of health. The advance made by the study lies in its theoretical framework on comfort, and provides the health team with a scale based on empirical evidence.

ResumoObjetivo:validar a Escala de Conforto para Familiares de pessoas em estado crítico de saúde.Método:trata-se de estudo metodológico. A amostra foi constituída por 274 familiares de pessoas adultas internadas em seis unidades de terapia intensiva que responderam a 62 itens, distribuídos em 7 dimensões. Os procedimentos de validação adotados foram embasados nas técnicas da Teoria Clássica dos Testes.Resultados:a análise da dimensionalidade foi realizada por meio da análise por componentes principais, obtendo-se uma escala com 55 itens distribuídos em 4 fatores: segurança, suporte, interação familiar/ente e integração consigo e com o cotidiano. A análise do poder discriminativo dos itens, realizada pelo coeficiente de correlação item-total, mostrou boa relação dos itens com seus respectivos fatores. O exame da fidedignidade da escala, por meio da análise da consistência interna, apresentou coeficiente alfa de Cronbach elevado para os 4 fatores e a medida geral.Conclusão:a Escala de Conforto apresentou parâmetros psicométricos satisfatórios, constituindo-se no primeiro instrumento válido para a avaliação do conforto de familiares de pessoas em estado crítico de saúde. A pesquisa avançou na construção de um referencial teórico sobre o conforto, e disponibilizou à equipe de saúde uma medida pautada em evidências empíricas.

ResumenObjetivo:validar la Escala de Confort para Familiares de personas en estado crítico de salud.Método:se trata de un estudio metodológico. La muestra estuvo constituida por 274 familiares de personas adultas, internadas en seis unidades de terapia intensiva, que respondieron a 62 ítems, distribuidos en 7 dimensiones. Los procedimientos de validación adoptados fueron basados en las técnicas de la Teoría Clásica de las Pruebas.Resultados:el análisis de la dimensionalidad fue realizada por medio del análisis por componentes principales, obteniéndose una escala con 55 ítems distribuidos en 4 factores: seguridad, soporte, interacción familiar/ente e integración consigo y con lo cotidiano. El análisis del poder discriminatorio de los ítems, realizado por el coeficiente de correlación ítem-total, mostró buena relación de los ítems con sus respectivos factores. El examen de confiabilidad de la escala, realizado por medio del análisis de consistencia interna, presentó un coeficiente Alfa de Cronbach elevado para los 4 factores y la medida general.Conclusión:la Escala de Confort presentó parámetros psicométricos satisfactorios, constituyéndose en el primer instrumento válido para la evaluación del confort de familiares de personas en estado crítico de salud. La investigación avanzó en la construcción de un referencial teórico sobre el confort, y suministró al equipo de salud una medida guiada en evidencias empíricas.

The efficacy of 2-nitrovinylfuran derivatives against Leishmania in vitro and in vivo

Despite recent advances in the treatment of some forms of leishmaniasis, the available drugs are still far from ideal due to inefficacy, parasite resistance, toxicity and cost. The wide-spectrum antimicrobial activity of 2-nitrovinylfuran compounds has been described, as has their activity against Trichomonas vaginalis and other protozoa. Thus, the aim of this study was to test the antileishmanial activities of six 2-nitrovinylfurans in vitro and in a murine model of leishmaniasis. Minimum parasiticide concentration (MPC) and 50% inhibitory concentration (IC50) values for these compounds against the promastigotes of Leishmania amazonensis, Leishmania infantum and Leishmania braziliensis were determined, as were the efficacies of two selected compounds in an experimental model of cutaneous leishmaniasis (CL) caused by L. amazonensis in BALB/c mice. All of the compounds were active against the promastigotes of the three Leishmania species tested. IC50 and MPC values were in the ranges of 0.8-4.7 µM and 1.7-32 µM, respectively. The compounds 2-bromo-5-(2-bromo-2-nitrovinyl)-furan (furvina) and 2-bromo-5-(2-methyl-2-nitrovinyl)-furan (UC245) also reduced lesion growth in vivo at a magnitude comparable to or higher than that achieved by amphotericin B treatment. The results demonstrate the potential of this class of compounds as antileishmanial agents and support the clinical testing of Dermofural(r) (a furvina-containing antifungal ointment) for the treatment of CL.

Whole-body diffusion-weighted magnetic resonance imaging versus FDG-PET/CT for initial lymphoma staging: systematic review on diagnostic test accuracy studies / Ressonância magnética de corpo total com difusão comparada à FDG-PET/CT no estadiamento inicial do linfoma: revisão sistemática de estudos de acurácia diagnóstica

CONTEXT AND OBJECTIVE: Positron emission tomography with [18]F-fluoro-2-deoxyglucose (FDG-PET/CT) has been advocated as the method of choice for lymphoma staging, since it enables whole-body analysis with high sensitivity for detection of affected areas and because it combines capacities for anatomical and functional assessment. With technological advances, magnetic resonance imaging (MRI) has emerged as an alternative to FDG-PET/CT. This systematic review with meta-analysis aimed to compare whole-body diffusion-weighted MRI (WB-MRI) with FDG-PET/CT for lymphoma staging. DESIGN AND SETTING: Systematic review on diagnostic test accuracy studies conducted at a public university. METHODS: The Medline, Scopus, Embase and Lilacs databases were searched for studies published up to September 2013 that compared WB-MRI and FDG-PET/CT for lymphoma staging. The reference lists of included studies were checked for any relevant additional citations. RESULTS: Six studies that evaluated the initial lymphoma staging in 116 patients were included. WB-MRI and FDG-PET/CT agreed in 90.5% of the cases (κ = 0.871; P < 0.0001). In most of the studies, when there was disagreement between the methods, WB-MRI overstaged in relation to FDG-PET/CT. The sensitivity of WB-MRI and FDG-PET/CT, in comparison with the clinical-radiological standard, ranged from 59 to 100% and from 63 to 100% respectively. CONCLUSION: WB-MRI is a highly sensitive method for initial lymphoma staging. It has excellent agreement with FDG-PET/CT and is a great alternative for managing lymphoma patients, without using ionizing radiation or an intravenous contrast agent. .

CONTEXTO E OBJETIVO: A tomografia por emissão de pósitrons com 2-[18F]-fluoro-2-deoxi-D-glicose (FDG-PET/CT) tem sido defendida como método de escolha para o estadiamento do linfoma por realizar o estudo do corpo inteiro com boa sensibilidade para detecção das áreas acometidas e por combinar as capacidades de avaliação anatômica e funcional. Com os avanços tecnológicos, a ressonância magnética tem se apresentando como alternativa à FDG-PET/CT. Esta revisão sistemática com metanálise visa comparar a ressonância magnética de corpo inteiro (WB-MRI) com difusão com a FDG-PET/CT no estadiamento do linfoma. TIPO DE ESTUDO E LOCAL: Revisão sistemática de estudos de acurácia diagnóstica conduzida em universidade pública. MÉTODOS: Foi conduzida uma busca nos bancos de dados Medline, Embase, Scopus e Lilacs por estudos publicados até setembro de 2013 comparando a WB-MRI com a FDG-PET/CT no estadiamento do linfoma. As referências bibliográficas dos estudos incluídos foram checadas com a finalidade de encontrar citações adicionais relevantes. RESULTADOS: Foram incluídos seis estudos que avaliaram o estadiamento inicial do linfoma de 116 pacientes. A WB-MRI e a FDG-PET/CT concordaram em 90,5% dos casos (κ = 0,871; P < 0,0001). Na maioria dos estudos, quando houve discordância, a WB-MRI estabeleceu estadiamento superior à FDG-PET/CT. A sensibilidade da WB-MRI e da FDG-PET/CT, em relação ao padrão clínico-radiológico, variou de 59% a 100% e de 63% a 100%, respectivamente. CONCLUSÃO: A WB-MRI apresenta alta sensibilidade no estadiamento inicial do linfoma, excelente concordância com a FDG-PET/CT e representa uma ótima alternativa no manejo de pacientes com linfoma, sem utilizar radiação ionizante ou meio de contraste intravenoso. .

Costo-efectividad de 18FDG-PET/CT vs CT al final del tratamiento en pacientes pediátricos con Linfoma Hodgkin / 18FDG-PET/CT cost-effectiveness compared to CT at the end of treatment in pediatric Hodgkin's lymphoma patients

Objetivo Estimar la costo-efectividad de 18FDG-PET/CT comparado con CT seguido de 18FDG-PET/CT como prueba confirmatoria de un caso positivo en la evaluación al final del tratamiento en pacientes menores de 18 años con Linfoma Hodgkin (LH). Métodos Se construyó un árbol de decisión donde se comparó el uso de 18FDG-PET/CT con CT seguido de 18FDG-PET/CT como prueba confirmatoria de un caso positivo en la detección de lesión residual. El resultado se midió en Años de Vida Ganados (AVG). Se calculó la razón de costo-efectividad incremental. Se utilizó como umbral 3 veces el PIB per cápita por año AVG. Valores expresados en pesos colombianos de 2010 (1 US dólar = $ 1 897,89) Se realizaron análisis de sensibilidad univariados, bivariados y probabilísticos. Resultados Suponiendo un diferencial en AVG entre verdaderos positivos y falsos negativos de 13 meses, el costo de un AVG adicional con 18FDG-PET/CT comparado con CT seguido de 18FDG-PET/CT como prueba confirmatoria de un caso positivo en la evaluación al final del tratamiento en pacientes pediátricos con LH fue $ 34 508 590. Conclusión Si el diferencial de esperanza de vida entre verdaderos positivos y falsos negativos es de al menos un 1,03 años, el uso de 18FDG-PET/CT en la evaluación al final del tratamiento de pacientes pediátricos con LH, es una estrategia costo-efectiva para Colombia.

Objective Estimating the cost-effectiveness of 18FDG-PET/CT (positron emission tomography) compared to computer tomography (CT) followed by 18FDG-PET/CT as a confirmatory test for a positive case at the end of treatment in Hodgkin's lymphoma (HL) patients under 18 years-old. Methods A decision tree was built for comparing 18FDG-PET/CT to CT followed by 18FDG-PET/CT as a confirmatory test for a positive case in detecting residual lesions; outcome was measured in life years gained (LYG). The cost-effectiveness ratio was calculated; the threshold was 3 times the per capita GDP per LYG. Values were expressed in Colombian pesos for 2010 (1 US dollar=$ 1,897.89) and submitted to deterministic and probabilistic sensitivity analysis. Results Assuming a difference of 13 months in true positives' life expectancy compared to that for false negatives, the cost of an additional LYG with 18FDG-PET/CT compared to CT followed by 18FDG-PET/CT as a confirmatory test for a positive case when evaluating the end of pediatric HL patients' treatment was $ 34,508,590 (COP). Conclusion If differential life-expectancy between true positives and false negatives is at least 1.03 years, then using 18FDG-PET/CT for evaluating the end of HL pediatric patients' therapy is a cost-effective strategy for Colombia.

Linfomas asociados a infección por VIH en pacientes del Hospital Clínico San Borja Arriarán / Fundación Arriarán2001-2008 / Lymphomas associated with HIV infection in patients at the Hospital San Borja Arriarán /Fundación Arriarán 2001-2008, Santiago, Chile

Introduction: Hodgkin lymphomas (HL) and non Hodgkin lymphomas (NHL) are frequently associated to acquired immunodeficiency syndrome in adults. Objective: To systematize the clinical features and histological characteristics of lymphomas in AIDS patients, its treatment and outcomes in our institution. Patients and Methods: Retrospective analysis of patients with HIV-associated lymphoma between January 2001 and December 2008 at the San Borja Arriarán Hospital complex. Results: Information was obtained from 30 patients with NHL and 7 with HL, with a median of 40 years. The majority of tumors were Burkitt lymphoma (47%), diffuse large cell lymphoma B-cell (37%) and NHL of T lineage (10%). There was no CNS or cavities lymphoma. Almost all patients (86.7%) with NHL were treated with CHOP chemotherapy, 57% of those receiving treatment had progression or relapse from complete remission. A rescue chemotherapy was indicated in 4 patients. 73% of patients receiving CHOP, complete 5 to 6 cycles of chemotherapy. The use of CHOP chemotherapy for the subgroup of patients with Burkitt lymphoma achieved low rates of complete remission and frequent relapse and disease progression, showing that CHOP was ineffective in improving survival, especially in high risk patients. We found statistically significant differences in survival according to IPIae (International prognostic Index age-adjusted). Conclusion: Non-Hodgkin lymphoma in HIV patients treated with chemotherapy protocols PAlNDA persists in our environment as a disease with a poor prognosis compared with findings in the international literature. The incorporation of new drugs of proven utility as rituximab and specific schemes chemotherapy could improve these results. The establishment of prognostic groups established by IPIae can guide clinical work for the use of chemotherapy tailored to their specific risk and optimized according to histological type.

Introducción: Los linfomas de Hodgkin (LH) y no Hodgkin (LNH) se asocian con alta frecuencia al síndrome de inmunodeficiencia humana en adultos. Objetivo: Sistematizar los aspectos clínicos e histológicos de los linfoma que afectan a pacientes con SIDA, su tratamiento y resultados globales en nuestra institución. Pacientes y Métodos: Análisis retrospectivo de pacientes con linfoma asociado a VIH entre enero de 2001 y diciembre de 2008 en el complejo hospitalario San Borja Arriarán. Resultados: Se obtuvo información de 30 pacientes con LNH y 7 LH, con una mediana de 40 años. Los tipos histológicos predominantes fueron linfoma de Burkitt (47 %), linfoma difuso de células grandes de estirpe B (37 %) y LNH de estirpe T (10%). No se diagnosticaron LNH del SNC ni linfoma de cavidades. Casi la totalidad de los pacientes (86,7%) con LNH se trataron con esquema CHOP, 57% de quienes recibieron tratamiento presentaron progresión o recaída desde remisión completa, ofreciéndoles una quimioterapia de rescate a cuatro pacientes. El 73% de los pacientes que recibieron CHOP lograron completar entre cinco y seis ciclos de quimioterapia. El uso de quimioterapia CHOP para el subgrupo de pacientes con linfoma de Burkitt alcanzó bajos porcentajes de remisión completa y mayoritariamente progresó la enfermedad, siendo esta quimioterapia, inefectiva para mejorar la sobrevida, especialmente en los pacientes de riesgo alto. Se encontraron diferencias estadísticamente significativas en sobrevida según el IPIae (índice internacional pronóstico ajustado por edad) al ingreso. Conclusión: El LNH en los pacientes con VIH tratados con los protocolos de quimioterapia PANDA persiste en nuestro medio como una enfermedad de muy mal pronóstico comparado con los resultados en la literatura internacional. La incorporación de nuevos fármacos de demostrada utilidad como rituximab y esquemas específicos de quimioterapia podrían mejorar estos resultados. El establecimiento de grupos pronósticos establecidos por IPIae puede orientar el trabajo clínico para el uso de quimioterapia ajustada a su riesgo específico y optimizado según tipo histológico.

Conceptos cambiantes en los linfomas / Changes in the lymphoma concepts

Los conceptos y la clasificación de los linfomas se han modificado a través de los años como resultado de los avances tecnológicos en el estudio de las neoplasias linfo-hematopoyéticas. Los linfomas desde mediados del siglo pasado se han clasificado en dos grupos: 1. Enfermedad de Hodgkin y 2. Linfomas no-Hodgkin; con sus tipos arquitectónicos y citológicos caracterizados con el microscopio de luz convencional. Sin embargo, la incorporación progresiva de técnicas de laboratorio especializada, principalmente inmunocito/histoquímicas y biología molecular, han permitido la identificación de nuevas variedades clínico-patológicas, inmuno-fenotípicas y genotípicas; de gran importancia terapéutica y pronóstica para los pacientes. En la actualidad, los linfomas se clasifican siguiendo las recomendaciones más recientes de la Organización Mundial de la Salud

The concepts and classification of the lymphomas have been modified throughout the years, as a result of the technological advances in the study of the lymphhematopoietic neoplasms. The lymphomas, since the middle of the last century have been classified in two groups: 1. Hodgkin´s disease, and 2. Non-Hodgkin’slymphomas; with its architectural and cytological types characterized with the conventional use of light microscope. Nevertheless, the progressive incorporation of specialized laboratory techniques mainly immunocyto-histochemestry and the molecular biology has allowed the identification of new clinic pathological, immuno-phenotypical and genotypic varieties; of great importance in the treatment and prognosis of the patients. At the present time, the lymphomas are classified following the most recent WorldHealth Organization recom mendations

Linfoma relacionado a infección por virus de la inmunodeficiencia humana en un hospital público de Santiago, Chile / HIV-related lymphoma in a public hospital in Chile. Analysis of 55 cases

Background: Cancer is the third cause of death in patients infected with human immunodeficiency virus (HIV) and lymphoma is the most common type. Aim: To describe the clinical characteristics, histology, risk factors and prognosis of these patients, in a Chilean public hospital in Chile. Material and Methods: Records of 55 patients (45 males) aged between 23 and 67years with lymphoma and HIV positive serology, diagnosed between 1992-2008, were reviewed. Results: Six patients (11%) had Hodgkin lymphoma (HL) and the rest, non-Hodgkin lymphoma (NHL). B-cell phenotype constituted 83.7% of NHL cases. The most common subtypes of all the lymphoma were diffuse large B cell lymphoma in 24 cases (43.6%), Burkitt lym-phoma in 12 cases (21.8%), andplasmablastic lymphoma in 5 cases (9.1%). Thirty five patients (64%) underwent curative intended chemotherapy (CT) concomitantly with highly active antiretroviral therapy (HAART). Three year survival of the whole cohort was 27%. By multivariate analysis, the most important prognostic factors for long term survival, were complete responses to CT, (p < 0.01) and a low international prognostic index (IPI) score for NHL, (p = 0.01). HAART, histologic subtype and CD4 lymphocyte count at diagnosis, did not influence survival. Conclusions: The most important prognostic factors for HIV patients with lymphoma, were achieving CR with CT and low IPI score. Prognosis remains poor, even with HAART therapy.

Síndrome de Sweet associada a linfoma de Hodgkin: relato de caso / Sweet's Syndrome associated with Hodgkin's disease: case report

A síndrome de Sweet é enfermidade cutânea rara e de etiologia pouco esclarecida. Cerca de 20 por cento dos casos são associados a neoplasias hematológicas, sendo raros os casos relacionados à doença de Hodgkin. Relata-se caso de paciente masculino de 57 anos que desenvolveu a síndrome concomitantemente à neoplasia. As doenças foram controladas com o tratamento específico.

Sweet's syndrome is a rare cutaneous disease of unknown etiology. About 20 percent of the cases are associated with hematological neoplasms, and cases related with Hodgkin's disease are rare. We present the case of a 57-year old male patient who developed the syndrome concomitantly with the neoplasm. The diseases were controlled with specific treatment.

Neoplasias sólidas de células redondas en edad pediátrica: correlación entre diagnóstico morfológico e inmunohistoquímica / Solid neoplasm of circle celis in the pediatric age: correlation between morphological and immunohistochemistry diagnóstic

Precisar la importancia de realizar el estudio de inmunohistoquímica y su correlación con el diagnóstico anatomopatológico de rutina para el diagnóstico de los tumores sólidos malignos de células redondas en pacientes pediátricos. Se realizó un estudio transversal, descriptivo, retrospectivo no experimental. Se estudiaron 147 pacientes con diagnóstico de neoplasia de células redondas, de los cuales 96 cumplieron con criterios de inclusión. La mayor frecuencia de casos la obtuvo el linfoma No Hodgkin (20,8%), seguido del linfoma de Hodgkin (15,6%) y tumor de Wilms (17,7%). El grupo etario mayormente afectado fue el escolar (37,5%). El género predominante fue el masculino (60,4%). Las manifestaciones clínicas más frecuentes fueron masa palpable (76,04%), fiebre(44,79%), anemia (41,67%), dolor (41,30%) y pérdida de peso (33,33%). Se evidenció la mayor correlación diagnóstica en el tumor de Wilms (100%), seguido del linfoma No Hodgkin (80%) y el linfoma de Hodgkin (75%); la menor correlación la presentaron el sarcoma de Ewingy el neuroblastoma (41,7%, ambos). La correlación total resultó “considerable” según la fuerza de concordancia del coeficiente de Kappa (k: 0,601. P=0,000). Se requiere la integración del examen histopatológico de rutina como método diagnóstico de pesquisa y del estudio de inmunohistoquímica como método auxiliar diagnóstico, para determinar la histogénesis de las neoplasias, descartar diagnósticos diferenciales, realizar un diseño terapéutico apropiado y precisar el pronóstico.

To precise the state and the importance o fthe study of immunohistochemistry and its correlation with the routine pathological diagnosis to use in diagnosis of malignant solid tumors of round cells in pediatric patients. We was performed a transversal, descriptive and retrospective non-experimental study. We studied 147 patients diagnosed with round cell tumor, 96 of them weremet in inclusion criteria. The highest frequency of the cases was Non-Hodgkin lymphoma (20.8%), followed by the Hodgkin lymphoma (15.6%) and the Wilms tumor(17.7%). The age group most affected was the school(37.5%). The male gender was the predominant (60.4%).The most frequent clinical manifestations were a palpablemass (76.04%), the fever (44.79%), the anemia (41.67%),the pain (41.30%) and the weight loss (33.33%). Evidencedthe highest correlation in the diagnosis of Wilms tumor(100%), followed by Non-Hodgkin lymphoma (80%) and Hodgkin lymphoma (75%); showed the lowest correlation between the Ewing sarcoma and neuroblastoma (41.7%, both). The total correlation is “considerable” accordingto strength of agreement the Kappa coefficient (k: 0.601.P=0.000). It requires the integration of routine histopathological examination as diagnostic method of research and study of immunohistochemistry as an auxiliary diagnostic method to determine the histogenesis of neoplasm and exclude the differential diagnoses, make anappropriate therapeutic design and the determine prognosis of this group of patients.

Epidemiología del linfoma con compromiso de cabeza y cuello en el Centro Asistencial Sótero del Río / Epidemiology of lymphoma of the head and neck in the Centro Asistencial Sótero del Río

Introducción: Los linfomas son la neoplasia hematológica más frecuente del adulto, ocupando el tercer lugar de las neoplasias de cabeza y cuello (CyC). Se distinguen dos tipos, el linfoma de Hodgkin (LH) y el linfoma no Hodgkin (LNH), constituyendo dos entidades clínicas separadas. Objetivo: Evaluar las manifestaciones de los linfomas en la región de CyC, analizando las diferencias de presentación y comportamiento entre en LH y el LNH. Material y método: Estudio retrospectivo de todos los casos de linfomas de CyC en el Centro Asistencial Dr. Sótero del Roo (CASR) entre los años 2005 y 2009, documentando específicamente presentación clínica, factores de riesgo, el tipo de linfoma, diagnóstico histológico, localización anatómica, etapificación al momento del diagnóstico, tratamiento administrado, sobrevida. Resultados: Se incluyeron 80 casos de pacientes con linfoma de CyC en el estudio, 16 (20 por ciento) con LH y 64 (80 por ciento) con LNH. Se encontraron diferencias estadísticamente significativas entre ambos para la edad de diagnóstico, el compromiso extranodal, compromiso de mediastino, presencia de síntomas B y sobrevida. Conclusión: En el CASR la presentación de los linfomas es similar a la reportada mundialmente con diferencias similares entre LH y LNH.

Introduction: Lymphomas are the most common hematologic neoplasia in the adult and the third most common neoplasias of the head and neck (CyC). There are two types of lymphoma: Hodgkin and non-Hodgkin lymphomas, which constitute two unique clinical entities. Aim: To evaluate the clinical manifestations of lymphomas in the CyC region, analizing the diferences between the clinical presentation and behavior of LH and LNH. Material and method: This is an retrospective study of all the cases of lymphoma of CyC in the Centro Asistencial Dr. Sótero del Río (CASR) between the years 2005 and 2009, describing specifically the clinical presentation, risk factors, type of lymphoma, histologic diagnosis, anatomical localization, etapification at diagnosis, treatment received and survival. Results: Eighty cases of lymphoma of the CyC were included, 16 patients (20 percent) with LH and 64 patients (80 percent) with LNH. We found statistically significant differences between both groups for their age at diagnosis, extranodal disease, mediastinal compromise, presence of "B" symptoms and survival. Conclusion: in the CASR the clinical presentation of lymphomas is similar to that reported in the international literature, maintaining the unique characteristics for LH and LNH.

Anemia hemolítica autoinmune como manifestación inicial de linfoma Hodgkin / Auto inmune hemolytic anemia how initial manifestation of Hodgkin lymphoma

Se presenta el caso de una paciente de 66 años de edad, a la que se le diagnóstica anemia hemolítica, la cual fue refractaria al tratamiento y requirió esplenectomía. Además presenta adenomegalias inguinales, cuya biopsia determina infiltración parcial por células linfoides B CD20+, con atipia, y CD30+, con factor de proliferación alto; en médula ósea se constata incremento de linfocitos T. Cuatro meses después, consulta por la aparición de adenopatías inguinales y axilares, de las cuales la biopsia reveló enfermedad de Hodgkin variante esclerosis nodular, y en médula ósea se evidenció infiltración por la enfermedad linfoproliferativa. Si bien es infrecuente la asociación entre anemia hemolítica y linfoma Hodgkin, debe tenerse en cuenta para llegar a la búsqueda oportuna de su causa y al diagnóstico de un probable proceso linfoproliferativo subyacente.

In this work we report and study a case of 66 year old woman, whit diagnosis of hemolytic anemia, which was refractory to treatment and she required splenectomy. The patient presented inguinal lymphadenopathy which biopsy has determined a partial infiltration of B-cells CD20+ and CD30+ with atypia and high growth factor. The bone marrow biopsy informed an increased number of T lymphocytes. Four month later, the patient complained due to the appearance of inguinal and axillaries lymph nodes, which biopsy revealed a nodular sclerosis Hodgkin lymphoma. The bone marrow biopsy showed infiltration by lymphoproliferative disease. Although the association between hemolytic anemia and Hodgkin lymphoma is less frequent, this fact should be taken into account in searching its cause and reaching the diagnosis of a probable underlying lymphoproliferative process.

Osseous involvement in pediatric hodgkin’s lymphoma.

Presented here is a report of 3 children, out of 66 total pediatric Hodgkin’s lymphoma (HL) patients (4.5%) with bone involvement over a 3 and a half year period. Two patients presented with osseous lesions at the time of relapse and one had concurrent evidence of non-osseous disease. The clinical presentation, radiographic findings, histology, treatment and outcome of these patients are discussed. Boys and girls are nearly equally affected, local bone pain is the commonest symptom and B-symptoms are common. Vertebrae and pelvis are most frequently involved, commonly with an osteolytic picture. Nodular sclerosis is the predominant histological subtype. Most children received combined modality treatment and the overall response and survival are good.

Linfomas asociados con la infección por el virus de la inmunodeficiencia humana: subtipos histológicos y asociación con los virus de Epstein Barr y Herpes-8 / AIDS related lymphomas: Histopathological subtypes and association with Epstein Barr virus and Human Herpes virus type-8

Los linfomas no Hodgkin (LNH) constituyen la segunda neoplasia definitoria de Sida más frecuente. En el presente trabajo se evaluaron 48 casos de linfomas asociados con la enfermedad debida al virus de la inmunodeficiencia humana (HIV) diagnosticados en la División Histopatología del Instituto de Investigaciones Hematológicas de la Academia Nacional de Medicina. Se incluyeron en la investigación 5 mujeres y 43 hombres con una mediana de edad al momento del diagnóstico de la neoplasia de 37 años. La evaluación morfológica se realizó en cortes coloreados con hematoxilina-eosina, estudio inmunohistoquímico para la detección del virus de Epstein Barr (VEB) en 48/48 casos, y mediante sonda oligonucleotídica biotinilada para la detección del ADN del Herpes virus humano tipo-8 (HHV-8) en 14/14 linfomas plasmoblásticos (LP). Todos fueron linfomas de fenotipo B, con un curso clínico agresivo y enfermedad neoplásica avanzada al momento del diagnóstico. Se pudo demostrar la fuerte asociación del VEB con los linfomas asociados al sida, con frecuencias que variaron según el subtipo histológico: 16/21 (76%) para los linfomas difusos de grandes células; 1/3 casos (33%) de linfomas de Burkitt y 3/4 (75%) en los linfomas primarios del sistema nervioso central. Globalmente, el genoma del VEB se detectó en 20/28 (71%) de las muestras de biopsias de LNH de esta serie. La detección del HHV-8 resultó negativa en los 14 LP. Los linfomas de Hodgkin fueron más frecuentes en varones,18/20 (90%), con un curso clínico agresivo y franco predominio de los subtipos histológicos de peor pronóstico (90% de casos). En estas neoplasias también se comprobó una frecuente asociación patogénica con el VEB (90% de casos).

Non-Hodgkin lymphomas (NHL) of the B-cell type are the second most common neoplasm among patients with human immunodeficiency virus (HIV) infection and AIDS. Here, we evaluated 48 cases of AIDS-related lymphomas (ARL) diagnosed at the Histopathological Division of the Instituto de Investigaciones Hematológicas of the National Academy of Medicine. Five were females and 43 were males with a median of age of 37 years at the time of the diagnosis. Micrometer sections were prepared and stained with hematoxilin-eosin; immunohistochemical examination for the presence of Epstein-Barr virus (EBV) was carried out in 48/48 cases. Additionally, biotinilated oligonucleotides were used to determine the presence of DNA of the Human Herpes virus type-8 (HHV-8) in 14/14 biopsy smears corresponding to plasmablastic lymphomas (PL). All were fenotype B cell lymphomas with an aggressive course and advanced neoplasm disease at the time of diagnosis. Virological findings showed the strong association between EBV and AIDS-related NHL. According to the histopathological subtype, the EBV genome was detected in 16/21 (76%) diffuse large B cell lymphomas, 1/3 Burkitt lymphoma and 3/4 (75%) of primary central nervous system lymphomas. Globally, EBV genome was detected in 20/28 NHL of this series. Detection of HHV-8 was negative in all cases of PL. Hodgkin lymphoma were more frequent in males 18/20 (90%), with an aggressive clinical course and a significant predominance of the subtypes associated with worse prognosis (90% of cases). We detected a significant association between EBV and HL (90% of cases). We consider that all cases of AIDS related lymphomas should be assessed for the presence of EBV because its presence may play a role in the prognosis.