Adenopatías gigantes en paciente con diagnóstico de linfoma no Hodking / Giant Lymphadenopathy in a Patient Diagnosed with Non-Hodgkin's Lymphoma

El linfoma se encuentra en el área de los ganglios linfáticos a ambos lados (superior e inferior) del diafragma, así como en el bazo(AU)

Síndrome colestásico como forma de presentación de linfoma no Hodgkin. Reporte de un caso pediátrico / Cholestatic syndrome as a presentation of non-Hodgkin lymphoma. A pediatric case report

La ictericia colestásica se debe a la alteración de la secreción de bilirrubina conjugada; es una de las posibles causas la alteración del flujo biliar por obstrucción de la vía biliar extrahepática. El linfoma es la tercera neoplasia más frecuente en pediatría, mientras que los tumores pancreáticos son poco frecuentes y, en su mayoría, lesiones benignas. Las manifestaciones clínicas de los tumores de localización retroperitoneal son poco específicas y suelen ser tardías, por lo que la sospecha clínica debe ser alta. El objetivo del siguiente trabajo es presentar el caso de un niño de 7 años con síndrome colestásico en el que se halló un tumor en la cabeza del páncreas que comprimía la vía biliar extrahepática. El diagnóstico del tumor fue linfoma no Hodgkin (LNH). Se destaca la infrecuencia de este tumor en esta localización en la edad pediátrica

Cholestatic jaundice is due to an alteration in conjugated bilirubin secretion; a possible cause is an altered bile flow resulting from an obstruction of the extrahepatic bile duct. A lymphoma is the third most common neoplasm in pediatrics, while pancreatic tumors are rare and mostly benign. The clinical manifestations of retroperitoneal tumors are not very specific and are usually late, so a high level of clinical suspicion is required. The objective of this study is to describe the case of a 7-year-old boy with cholestatic syndrome with a tumor in the head of the pancreas compressing the extrahepatic bile duct. The tumor diagnosis was non-Hodgkin lymphoma (NHL). It is worth noting that the presence of a tumor in this location in pediatric age is uncommon

Linfoma no Hodgkin: primera y segunda neoplasia o, transformación histológica: dificultad diagnóstica: reporte de un caso / Non-Hodgkin's lymphoma: first and second neoplasia or, histological transformation: diagnostic difficulty: a case report

El LF es una enfermedad heterogénea con muchos diferentes subgrupos, en términos de edad de inicio, órganos involucrados (especialmente sitios extranodales) y anormalidades genéticas. Con grandes variaciones en el curso clínico, algunas veces muy indolente o no; puede mostrar transformación a linfomas de alto grado, se han reportado transformaciones a Linfoma de Células Grandes B Difusas (siendo este el más común), de Burkitt, Leucemia/Linfoma, Linfoma Linfoblástico (reportado en raras ocasiones), con un curso clínico agresivo. En el 2008 la OMS estableció 3 nuevas variantes: 1) LF Pediátrico, 2) LF Primario Intestinal y 3) LF in situ. Patólogos y clínicos debemos considerar estas patologías cuando se decide el diagnóstico y el tratamiento. La transformación histológica se refiere al evento biológico que lleva al desarrollo de un Linfoma No Hodgkin agresivo de alto grado en pacientes con un LF subyacente. El Linfoma Folicular (LF) pediátrico es una neoplasia excesivamente rara, que difiere del LF del adulto.

FL is a heterogeneous disease with many different subgroups, in terms of age of onset, organs involved (especially extranodal sites) and genetic abnormalities. With great variations in the clinical course, sometimes very indolent or not; can show transformation to high-grade lymphomas, transformations to Diffuse Large B Cell Lymphoma (this being the most common), Burkitt's, Leukemia / Lymphoma, Lymphoblastic. Lymphoma (reported rarely), with an aggressive clinical course have been reported. In 2008 the WHO established 3 new variants: 1) Pediatric FL, 2) Intestinal Primary FL and 3) in situ FL. Pathologists and clinicians must consider these pathologies when deciding on diagnosis and treatment. Histological transformation refers to the biological event that leads to the development of high-grade aggressive Non-Hodgkin's Lymphoma in patients with an underlying FL. Pediatric Follicular Lymphoma (FL) is an excessively rare neoplasm, which differs from adult FL.

Pérdida de agudeza visual progresiva en paciente con proptosis. Caso clínico y revisión de la literatura / Progressive visual impairment in a patient with proptosis. Case report and review of the literature.

Introducción: a raíz del siguiente reporte de caso clínico se pretende repensar el diagnóstico diferencial de los tumores orbitales y revisar la literatura existente al respecto. Caso: paciente de 54 años, fumadora, acude a nuestro centro por una pérdida de agudeza visual progresiva de dos años de evolución en el ojo derecho, que se acompañaba de proptosis. Las pruebas de imagen basadas en resonancia magnética y tomografía por emisión de positrones ­ tomografía computarizada (PET-TC) realizadas describían una lesión intraconal derecha de morfología indefinida, que rodeaba el nervio óptico. El estudio inmunohistoquímico y molecular anatomopatológico confirmó la sospecha de síndrome linfoproliferativo extranodal de bajo grado. Discusión: el manejo endoscópico de estas lesiones puede resultar en una menor comorbilidad en comparación con el abordaje externo tradicional. El papel de la cirugía radica en la obtención de una muestra de la lesión que permita un correcto diagnóstico. Conclusiones: el abordaje multidisciplinar con oftalmólogos, hematólogos y expertos en radioterapia permite obtener buenos resultados quirúrgicos y clínicos en la inmensa mayoría de casos.

Introduction: as result of the following clinical case report, we intend to review the differential diagnosis of orbital tumors and review the existing literature in this regard. Case report: a 54-year-old smoking patient, consulted to our department due to a progressive visual impairment over the last two years in her right eye. She presented proptosis in her clinical examination. Imaging studies based on MRI and PET-CT described a right intraconal lesion with an undefined morphology surrounding the optic nerve. Orbital tumors differential diagnosis is delicate. Nevertheless, Non-Hodgkin lymphomas followed by metastasis are the two most common found in this location. The immunohistochemistry and molecular studies, confirmed the suspected diagnosis of extranodal low-grade lymphoproliferative syndrome. Discussion: endoscopic management of these lesions may result in a lower comorbidity compared to traditional external approaches. Role of surgery lays in obtainment of a quality sample which allows a proper diagnosis. Conclusions: multidisciplinary approach with ophthalmologists, hematologists and radiotherapy experts enhance good surgical and clinical results in the vast majority of cases.

Linfoma no Hodgkin localizado en el tejido mamario. presentación de un caso / Non-Hodgkin lymphoma located in the breast tissue. presentation of a case

Se presentó el caso de una adolescente de 12 años de edad que acudió a consulta externa de Cirugía del Hospital Pediátrico Docente "Gral. Pedro Agustín Pérez", de Guantánamo; la misma presentó aumento de volumen a nivel del cuadrante superior externo de la mama izquierda, no doloroso. Se palpó tumoración de bordes irregulares, o sea mal definidos, de 3 cm de diámetro aproximadamente, sin signos de inflamación. Se realizó biopsia por aspiración con aguja fina y se informó el diagnóstico de fibroadenoma de mama. La exéresis del nódulo y el estudio histopatológico demostraron el diagnóstico de linfoma no Hodgkin de alto grado de malignidad y se procedió a la atención multidisciplinaria. Al momento del reporte la paciente continúa con quimioterapia y evoluciona satisfactoriamente. Se revisó la literatura médica sobre el tema y se expusieron sus bases esenciales para la familiarización de los médicos generales(AU)

A 12 years old young female presented to the surgical outpatient clinic at the Hospital Pediátrico Docente "Gral. Pedro Agustín Pérez" in Guantanamo. Patient showed swelling in the upper right breast, with no pain. Examination revealed irregular edges (with approximately 3 cm in diameter) and no swollen tissue. Fine-needle aspiration biopsy was performed and breast fibroadenoma was diagnosed. Node exeresis and histopathological study both showed a high risk malignant non-Hodgkin's lymphoma, and a multidisciplinary care team assumed the case. At the time of the report, the patient is still under chemotherapy and prognosis evolves positively. The medical literature on the subject was put on review and a layout of the essentials to familiarize the general practitioners with the topic was set(AU)

Supervivencia de pacientes con linfoma no-Hodgkin y síntomas neurológicos según inmunofenotipo del líquido cefalorraquídeo / Survival in non-Hodgkin´s lymphoma patients with neurological symptoms according to the cerebrospinal fluid immunophenotype

Introducción: Los linfomas no-Hodgkin pueden infiltrar el sistema nervioso central y producir síntomas neurológicos, lo cual incrementa la mortalidad. El diagnóstico de esta infiltración se puede realizar mediante el estudio del líquido cefalorraquídeo por la técnica de citometría de flujo, con una mayor sensibilidad que la citología convencional. Objetivo: Estimar la supervivencia global de pacientes con Linfoma no-Hodgkin y síntomas neurológicos según el inmunofenotipo celular del líquido cefalorraquídeo. Métodos: Se realizó un estudio analítico y prospectivo en 15 pacientes con diagnóstico confirmado de linfoma no-Hodgkin y síntomas neurológicos, con citología negativa del líquido cefalorraquídeo, tratados en el servicio de oncología del Instituto Nacional de Oncología y Radiobiología, durante los años 2017 y 2018. El inmunofenotipo fue caracterizado mediante citometría de flujo multiparamétrica. Resultados: El 60,0 por ciento de los pacientes fue del sexo femenino y el 53,4 por ciento mayor de 60 años. Hubo una mortalidad del 26,7 por ciento. Se realizaron 17 inmunofenotipos, el 58,9 por ciento fue normal, el 23,4 por ciento reactivo y el 17,7 por ciento sospechoso de malignidad. La supervivencia global fue mayor en pacientes con líquido cefalorraquídeo con inmunofenotipo normal (HR. 0.04). Conclusiones: La citometría de flujo pudo discriminar células sospechosas de malignidad, en pacientes cuyas citologías fueron negativas. La presencia en el líquido cefalorraquídeo de células atípicas, de pleocitosis y de un índice de linfocito-monocito alto se asoció con una supervivencia global menor(AU)

Introduction: When non-Hodgkin lymphomas infiltrate the central nervous system increases mortality. The diagnosis of this infiltration can be made by the study of cerebrospinal fluid using flow cytometry, with a higher sensitivity than conventional cytology. Objective: To estimate the relationship between the cellular immunophenotype of the cerebrospinal fluid and the overall survival of patients with non-Hodgkin lymphoma and neurological symptoms. Methods: An analytical and prospective study was conducted in 15 patients with confirmed diagnosis of non-Hodgkin lymphoma and neurological symptoms, with negative cytology of the cerebrospinal fluid. Patients cared at Oncology Department of the National Institute of Oncology and Radiobiology, during the years 2017-2018. The immunophenotype was characterized by multiparametric flow cytometry. Results: 60.0 percent of the patients was female and 53.4 percent older than 60 years. There was an overall mortality of 26.7 percent 17 immunophenotypes were found, 58.9 percent of them was normal, 23.4 percent reactive and 17.7 percent suspected of malignancy. Overall survival advantage was obtained in patients with cerebrospinal fluid with normal immunophenotype (HR 0.04). Conclusions: Flow cytometry could discriminate cells suspected of malignancy, in patients whose cytologies were negative. The presence in the cerebrospinal fluid of atypical cells, pleocytosis and a high lymphocyte-monocyte index were associated with a lower overall survival(AU)

Pediatric abdominal non-Hodgkin's lymphoma: diagnosis through surgical and non-surgical procedures / Linfoma não Hodgkin abdominal pediátrico: diagnóstico por meio de procedimentos cirúrgicos e não cirúrgicos

Abstract Objective: To describe the success rate and the complications after procedures to diagnose abdominal non-Hodgkin's lymphoma in children and adolescents. Methods: A retrospective cross-sectional study was conducted with a population consisting of children and adolescents with abdominal non-Hodgkin's lymphoma diagnosed between September 1994 and December 2012. The sample comprised of 100 patients who underwent 113 diagnostic procedures, including urgent surgery (n = 21), elective surgery (n = 36), and non-surgical diagnosis (n = 56). Results: The most frequent procedures were laparotomy (46.9%) and ultrasound-guided core biopsy (25.6%). The rate of diagnostic success was 95.2% for urgent surgeries; 100% for elective surgeries and 82.1% for non-surgical procedures (p < 0.05). The rates of complication during the three diagnosis procedures considered were significant (p < 0.001; 95.2% of the urgent surgeries, 83.8% of the elective surgeries, and 10.7% of the non-surgical procedures). The length of time before resuming a full diet and starting chemotherapy was significantly reduced for patients who underwent non-surgical procedures when compared with the other procedures (p < 0.001). Conclusion: Non-surgical procedures for the diagnosis of pediatric abdominal non-Hodgkin's lymphoma are an effective option with low morbidity rate, allowing an earlier resumption of a full diet and chemotherapy initiation. Furthermore, non-surgical procedures should also be considered for obtaining tumor samples from patients with extensive disease.

Resumo Objetivo: Descrever a taxa de sucesso e as complicações dos procedimentos para o diagnóstico de linfoma não Hodgkin abdominal em crianças e adolescentes. Métodos: Estudo retrospectivo transversal em uma população de crianças e adolescentes com linfoma não Hodgkin abdominal diagnosticada entre setembro de 1994 e dezembro de 2012. A amostra foi composta por 100 pacientes submetidos a 113 procedimentos diagnósticos, inclusive cirurgia de urgência (n = 21), cirurgia eletiva (n = 36) e diagnóstico não cirúrgico (n = 56). Resultados: Os procedimentos mais frequentes foram laparotomia (46,9%) e biópsia guiada por ultrassonografia (25,6%). A taxa de sucesso diagnóstico foi de 95,2% para cirurgias de urgência; 100% para cirurgias eletivas e 82,1% para procedimentos não cirúrgicos (p < 0,05). Houve diferença significativa entre as taxas de complicação associadas aos três grupos (p < 0,001; 95,2% das cirurgias urgentes, 83,8% das cirurgias eletivas e 10,7% dos procedimentos não cirúrgicos). O tempo decorrido até o reinício da dieta plena e o início a quimioterapia foi significativamente reduzido para os pacientes submetidos a procedimentos não cirúrgicos quando comparados com os outros procedimentos (p < 0,001). Conclusão: Os procedimentos não cirúrgicos para o diagnóstico do linfoma não Hodgkin abdominal pediátrico são uma opção efetiva com baixa taxa de morbidade, permitem uma retomada mais precoce de uma dieta plena e início de quimioterapia. Em pacientes com doença extensa, os procedimentos não cirúrgicos também devem ser considerados para a obtenção de amostras tumorais.

Oligoartritis, paniculitis y pioderma gangrenoso como manifestaciones de un Linfoma no Hodgkin / Oligoarthritis, panniculitis and pyoderma gangrenosum associated with non-Hodgkin lymphoma: report of one case

An assortment of clinical and laboratory abnormalities may occur as paraneoplastic syndromes in lymphomas. Rheumatological and dermatological manifestations such as paraneoplastic arthritis and pyoderma gangrenosum must be underscored. We report a 28 years old woman who developed pyoderma gangrenosum and two years later presented with arthritis of knees and ankles associated with panniculitis interpreted as erythema induratum that was pathologically confirmed. She developed a reactivation of pyoderma gangrenosum, that was refractory to treatment. Complementary studies showed a pulmonary nodule and a right paravertebral mass with involvement of the psoas muscle. Biopsies of both masses and a new pathological skin study demonstrated a large B-cell non-Hodgkin's lymphoma.

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

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

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

Linfoma primario difuso de células B grandes de mucosa gingival, en paciente VIH y EBV negativos / Primary gingival diffuse large b-cell lymphoma in HIV/EBR negative patient abstract

El linfoma difuso de células B grandes (LDCBG) es el linfoma más frecuente. La presentación clínica puede ser nodal o extranodal y sus síntomas dependen de la localización tumoral; en la mayoría de los casos están asociados a algún tipo de inmunodeficiencia. Referiremos un caso de LDCBG de presentación atípica en una localización muy infrecuente. Es importante tener en cuenta estas situaciones, ya que pueden simular otros procesos patológicos, retrasando así su correcto diagnóstico y por lo tanto un adecuado tratamiento. (AU)

Diffuse large cell lymphoma B (LDCBG) is the most common type of lymphoma. It´s clinical presentation can be nodal or extranodal and it's symptoms depend where the tumor is located and whether is associated or not with an immunodeficiency disease. We present an atypical presentation of a LDCBG in a very unusual location. It´s important to consider these kind of appearance, as they can mimic other oral pathological processes, delaying their correct diagnosis and therefore an appropriate treatment. (AU)

Linfoma no Hodgkin folicular primario del bazo / Follicular Non-Hodgkin Primary Splenic Lymphoma

Se presenta el caso de una paciente de 79 años que desde hace 3 meses presenta marcada sudoración nocturna, dolor abdominal en zona de hipocondrio y flanco izquierdo y ligera pérdida de peso. Al examen físico se observa palidez de piel y mucosas, esplenomegalia que rebasa 7 cm el reborde costal izquierdo, superficie lisa, superficie regular. El resto del examen físico fue normal. Se realizan múltiples investigaciones básicas sin poderse identificar el diagnostico. Por la presencia de gran esplenomegalia y la pancitopenia se decide realizar esplenectomía confirmándose un linfoma no Hogkin folicular primario del bazo(AU)

We present the case of a 79-year-old patient who had marked night sweats, abdominal pain in the hypochondrium area and left flank and slight weight loss for 3 months. Physical examination showed pale skin and mucous membranes, splenomegaly that exceeds the left costal margin by 7 cm, smooth surface, and regular surface. The rest of the physical examination was normal. Multiple basic studies were conducted out but the diagnosis was not identified. Due to the presence of great splenomegaly and pancytopenia, it was decided to perform splenectomy, confirming a follicular non-Hodgkin primary splenic lymphoma(AU)

Linfoma gástrico no Hodgkin perforado / Perforated gastric non-Hodgkin lymphoma

Los linfomas son tumores sólidos del sistema linfático y se subdividen en linfomas de Hodgkin y no-Hodgkin. Los tumores no Hodgkin pueden originarse en tejidos no ganglionares como el tubo digestivo y son considerados primarios cuando el compromiso extranodal es igual o superior al 75% con relación al compromiso nodal. Los linfomas extranodales gastrointestinales representan del 1% al 4% de los tumores del tracto digestivo y del 10 al 15% de todos los linfomas no Hodgkin. El estómago es el sitio extranodal más común de los linfomas. Presentamos el caso clínico de un paciente varón sexagenario natural de Chiclayo que se realiza una endoscopía particular por síntomas inespecíficos de dispepsia y sólo se informa gastritis eritematosa y al ser admitido en nuestra institución se le diagnostica por endoscopía, marcadores bioquímicos y anatomopatología como un linfoma gástrico no Hodgkin perforado

Lymphomas are solid tumors of the lymphatic system and these are divided into Hodgkin's and non-Hodgkin lymphoma. Non-Hodgkin tumors may originate from non-nodal tissues such as the gastrointestinal tract and they are considered primary when extranodal involvement is equal to or greater than 75% according to the nodal involvement. Extranodal gastrointestinal lymphomas represent 1% to 4% of tumors of the digestive tract, and 10 to 15% of all non-Hodgkin lymphomas. The stomach is the most common extranodal lymphomas site. We present the clinical case of a sexagenarian male natural of Chiclayo who had an upper GI endoscopy for non-specific symptoms of dyspepsia that only reports gastritis and when admitted to our institution is diagnosed by endoscopy, biochemical markers and pathology as a perforated gastric non-Hodgkin lynphoma

Granulomas pulmonares y en médula ósea: más allá de la tuberculosis. A propósito de un caso / Differential diagnosis of bone marrow and lung granulomas: Report of one case

The differential diagnosis of pulmonary tuberculosis and lymphoma with pulmonary infiltration is very difficult, given their similar clinical characteristics. We report a 59 year old female with weight loss, fever, dyspnea and cough of several months of duration. She had a cavitated mass in lung imaging. A positive conventional PCR lead to the diagnosis of tuberculosis, but she had negative smears and cultures for Mycobacterium tuberculosis. The patient did not respond to treatment and her clinical condition worsened. A peripheral lymph node biopsy confirmed the presence of a diffuse large B cell non-Hodgkin lymphoma. Bone marrow pathology showed non caseating granulomas, again with negative microbiological tests for M. tuberculosis. Findings in the bone marrow were interpreted as a secondary sarcoid reaction to cancer and PCR results as a false positive. The lymphoma was treated, achieving complete remission. This case highlights the importance of the differential diagnosis between these two entities.

Guía de Práctica Clínica para la detección, tratamiento y seguimiento de Linfomas Hodgkin y No Hodgkin en población mayor de 18 años / Clinical practice guideline for the detection, treatment and follow-up of Hodgkin and Non-Hodgkin lymphomas in a population over 18 years of age

Esta guía esta dirigida al personal de la salud involucrado directamente en la atención de pacientes adultos mayores de 18 años con sospecha o diagnóstico de linfoma no Hodgkin B difuso de célula grande (Linfoma B difuso de células grandes), linfoma folicular (LF), linfoma de células del manto (LCM) y linfoma Hodgkin (LH), y a las instancias administrativas, empresas aseguradoras y entes gubernamentales involucrados en la generación de políticas en salud. Esta GPC basada en la evidencia incluye los temas de diagnóstico y tratamiento del Linfoma B difuso de células grandes, LF, LM y LH, bajo la perspectiva del Sistema General de Seguridad Social en Salud colombiano. Objetivos: Determinar los métodos diagnósticos más apropiados en pacientes con LBDCG para garantizar un diagnóstico preciso que permita una adecuada selección del tratamiento; Establecer las líneas de tratamiento en pacientes con LF para disminuir la heterogeneidad en la atención y mejorar los resultados del tratamiento; Determinar los esquemas de tratamiento de primera línea para pacientes con LCM en diferentes grupos de edad para disminuir la heterogeneidad y mejorar los resultados del mismo; y Mejorar la supervivencia libre de enfermedad y la supervivencia global de los pacientes adultos con LH.

Immunohistochemical profile of Hodgkin and Non-Hodgkin lymphoma

Objective: To analyze the frequencies of histological types of lymphoma, diagnosed with complete immunohistochemical profile in younger and older age group

Study Design: Cross-sectional analytical study

Place and Duration of Study: Dow Diagnostic Research and Reference Laboratory, Dow University of Health Sciences, Karachi, from January 2009 to September 2013

Methodology: Consecutive cases of lymphomas, which were diagnosed using immunohistochemistry, were analyzed according to WHO classification. Frequency and percentages for different types of lymphomas were calculated. Hodgkin and non-Hodgkin lymphomas characteristics in two age groups of less than and more than 40 years were compared, applying chi-square test

Results: Out of the 318 cases, 79 [25%] were Hodgkin Lymphomas [HL] and 239 [75%] were Non-Hodgkin Lymphomas [NHL]. Mixed Cellularity Hodgkin Lymphoma [MCHL] was the commonest [n=48]. Amongst the NHL, 215 [89.95%] were B cell lymphomas and 24 [10.05%] were T-cell lymphomas. Diffuse Large B-Cell Lymphoma [DLBCL] was the commonest lymphoma [n=165, 69.95% of NHL]. Anaplastic T-Cell Lymphoma [ALCL, n=10] was the commonest T-cell lymphoma. The frequency of HL was significantly higher in the younger age group and that of NHL was higher in the older age group [p < 0.001]. Primary lymph node involvement was reported in 175 [55%] and cervical lymph node was the most frequent site. Extra nodal involvement was seen in 93 [29%] of all cases and was reported in 87 [36.4%] of NHL and 6 [7.5%] of HL. The most common extra nodal site was the gastrointestinal tract

Conclusion: Hodgkin lymphoma comprises 25% and non-Hodgkin lymphoma comprises 75% of all lymphomas. Both occur in younger age groups than reported in the West. B-cell NHL is three times more common than T-cell lymphoma. DLBCL is the most frequent lymphoma. ALCL is the most common T-cell, and mixed cellularity is the most common Hodgkin lymphoma

Comportamiento del linfoma no Hodgkin en la edad pediátrica / Behavior of non-Hodgkin lymphoma at pediatric ages

INTRODUCCIÓN: los linfomas no Hodgkin son neoplasias hematológicas frecuentes en la infancia. OBJETIVO: describir las diferentes formas de presentación de esta enfermedad en la edad pediátrica, y determinar el promedio de años vividos después de concluido el tratamiento. MÉTODOS: se realizó un estudio descriptivo retrospectivo en 79 pacientes con diagnóstico de linfomas no Hodgkin atendidos en el servicio de Oncocirugía del Hospital Pediátrico Docente "William Soler", de marzo de 1995 a marzo de 2014. RESULTADOS: 48 pacientes fueron del sexo masculino y 31 del femenino. El grupo de edad de mayor afectación fue el de 10 a 14 años. El 54,4 % de los pacientes tuvieron linfomas de localización abdominal y el 29,1 % fueron de localización mediastinal. Otros sitios afectados fueron la región cervical, la amígdala palatina y renal primaria en el 10,1, 3,8 y 2,6 % de los pacientes respectivamente. La variante histológica más frecuente fue el linfoma no Hodgkin inmunofenotipo B (75,9 %), seguido del linfoma no Hodgkin inmunofenotipo tipo T en el 21,5 % de los casos. El dolor abdominal y la masa tumoral palpable fue la manifestación clínica principal en el 64,5 %. El promedio de años vividos en el linfoma no Hodgkin de localización renal, cervical y amígdala palatina fue ligeramente superior (5,7 ± 0,3, 5,5 ± 1,8 y 5,2 ± 0,7 respectivamente). CONCLUSIONES: el linfoma no Hodgkin inmunofenotipo B de localización abdominal es el más frecuente. El dolor y el tumor abdominal son las manifestaciones clínicas principales, y los pacientes con linfomas no Hodgkin de la región cervical y amígdala palatina tienen mayor promedio de vida después de concluido el tratamiento.

INTRODUCTION: non-Hodgkin lymphomas are frequent hematological neoplasias in infancy. OBJECTIVE: to describe the different forms of presentation of this disease at the pediatric age and to determine the average years lived after the treatment. METHODS: a retrospective and descriptive study of 79 patients was conducted; they had been diagnosed as non-Hodgkin lymphoma cases and attended to at the oncologic surgery service of "William Soler" pediatric teaching hospital from March 1995 to March 2014. RESULTS: forty eight patients were males and 31 were females. The most affected age group was the 10-14 years-old one. In the group, 54.4 % of patients had lymphomas located in the abdominal region and 29.1 % in the mediastinal location. Other affected areas were cervical region, palatal tonsil and primary renal region in 10.1 %, 3.8 % and 2.6 % of patients, respectively. The most common histological variant was immunophenotype B non-Hodgkin lymphoma (75.9 %) followed by immunophenotype T non-Hodgkin lymphoma (21.5 %). Abdominal pain and palpable tumor mass was the main clinical manifestation in 64.5 % of cases. The average life years in non-Hodgkin lymphoma located in the renal region, cervical region and palatal tonsil was slightly higher (5.7±0.3, 5.5±1.8 and 5.2±0.7, respectively). CONCLUSIONS: the immunophenotype B non-Hodgkin lymphoma of abdominal location is the most common. Abdominal pain and tumors are the main clinical manifestations and the patients with non-Hodgkin lymphomas in the cervical region and the palatal tonsil show higher average life years after treatment.

Crioglobulina con propiedad crioaglutinina en un paciente con linfoma esplénico de zona marginal / Cryoglobulin with cold agglutinin properties in a patient with splenic marginal zone lymphoma / Crioglobulina com propriedade crioaglutinina em um paciente com linfoma esplênico de zona marginal

El objetivo del trabajo fue describir un caso de linfoma esplénico de zona marginal (LEZM) con un componente monoclonal que posee propiedades de crioglobulina y crioaglutinina, un hallazgo de muy baja frecuencia. Una paciente con LEZM padeció una anemia hemolítica autoinmune 5 años después del diagnóstico, con hematocrito de 0,15 L/L, hemoglobina 49 g/L, lactato deshidrogenasa 16,82 μkat/L, prueba de Coombs directa positiva con anti- IgG/C3d, bilirrubina total 90,6 μmol/L e indirecta de 58,1 μmol/L. No presentó evidencia clínica ni serológica de infección por VIH, hepatitis B ni C. El proteinograma sérico presentó un pico monoclonal de 14 g/L, con crioglobulinemia positiva a las 24 h, y un criocrito de 30%. La crioglobulina purificada fue de tipo I con un componente monoclonal IgM-lambda, coincidente al observado en suero. El suero, el eluato a 37 °C y la crioglobulina purificada de la paciente presentaron actividad de crioaglutinina con especificidad anti-I, fenómeno producido por la misma inmunoglobulina. El hallazgo de una crioglobulina con propiedades de crioaglutinina en pacientes con LEZM no ha sido descrito previamente en la bibliografía.

The aim of this paper is to describe a case of splenic marginal zone lymphoma (SMZL) with monoclonal component having properties of cryoglobulin and cold agglutinin, a finding of very low frequency. A patient with SMZL suffered autoimmune hemolytic anemia five years after diagnosis, with hematocrit 0.15 L/L, hemoglobin 49 g/L, lactate dehydrogenase 16.82 μkat/L, direct Coombs test with anti-IgG/C3d positive, total bilirubin 90.6 μmol/L and indirect 58.1 μmol/L. She presented no clinical or serological evidence of HIV, hepatitis B or C infection. Serum protein electrophoresis showed a monoclonal peak of 14 g/L, with positive cryoglobulinemia at 24 hours, and 30% cryocrit. Purified cryoglobulin was type I with a monoclonal IgM-lambda component coincident with that observed in serum. The patient serum, eluate at 37 °C and purified cryoglobulin showed cold agglutinin activity with anti-I specificity, phenomenon produced by the same immunoglobulin. The finding of a cryoglobulin with cold agglutinin properties in patients with SMZL has not been previously described in the literature.

O objetivo do trabalho foi descrever um caso de linfoma esplênico de zona marginal (LEZM) com um componente monoclonal com propriedades do crioglobulina e crioaglutinina, um achado de muito baixa frequência. Um doente com LEZM sofreu uma anemia hemolítica autoimune cinco anos após o diagnóstico, com hematócrito de 0,15 L/L, hemoglobina 49 g/L, lactato desidrogenase de 16,82 μkat/L, teste de Coombs direto positivo com anti-IgG/C3d, bilirrubina total 90,6 μmol/L e indireta 58,1 μmol/L. Não apresentou evidência clínica ou sorológica de infecção por HIV, hepatite B ou C. O proteinograma sérico mostrou um pico monoclonal de 14 g/L, com crioglobulinemia positiva 24 horas, e um criocrito de 30%. Crioglobulina purificada foi tipo I com o componente monoclonal IgM-lambda, coincidente com a observada no soro. O soro, o eluato a 37 ° C e a crioglobulina purificada do paciente mostraram atividade de crioaglutinina com especificidade anti-I, fenômeno produzido pela mesma imunoglobulina. O achado de uma crioglobulina com propriedades de crioaglutinina em pacientes com LEZM não foi previamente descrito na literatura.

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. .

Gastric Perforation Caused by Primary Gastric Diffuse Large B Cell Lymphoma / 대한소화기학회지

Spontaneous gastric perforation is a rare complication of gastric lymphoma that is potentially life threatening since it can progress to sepsis and multi-organ failure. Morbidity also increases due to prolonged hospitalization and delay in initiating chemotherapy. Therefore prompt diagnosis and appropriate treatment is critical to improve prognosis. A 64-year-old man presented to the emergency department with severe abdominal pain. Chest X-ray showed free air below the right diaphragm. Abdominal CT scan also demonstrated free air in the peritoneal cavity with large wall defect in the lesser curvature of gastric lower body. Therefore, the patient underwent emergency operation and primary closure was done. Pathologic specimen obtained during surgery was compatible to diffuse large B cell lymphoma. Fifteen days after primary closure, the patient received subtotal gastrectomy and chemotherapy was initiated after recovery. Patient is currently being followed-up at outpatient department without any particular complications. Herein, we report a rare case of gastric lymphoma that initially presented as peritonitis because of spontaneous gastric perforation.