Application of the Central Nervous System International Prognostic Index (CNS-IPI) score in daily practice: a retrospective analysis apart from the clinical trial at two centers in Brazil

ABSTRACT Introduction: The diffuse large B-cell lymphoma (DLBCL) is the most common type of non-Hodgkin lymphoma (NHL) and, despite all the progress in this field, central nervous system infiltration (CNSi) still occurs at an incidence of 2-10%. The objective of the present study was to evaluate the Central Nervous System International Prognostic Index (CNS-IPI) score in daily practice regarding the reproducibility in a heterogeneous cohort apart from a clinical trial. Methods: Primary DLBCL patients were eligible for this study, between January 2007 and January 2017. All patients were treated with rituximab-based chemotherapy, mostly R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine and prednisone). The CNSi was diagnosed by liquor (positive cytology and/or immunophenotype), computerized tomography, magnetic resonance image and/or fluorodeoxy-glucose-positron emission tomography, requested only in symptomatic patients when the CNSi was clinically suspected. The CNS-IPI was assessed by graphical comparison and calibration. Results: After applying the inclusion/exclusion criteria, 322 patients were available for the analysis. The median follow-up was 60 months and the median age was 58 years. Seven patients experienced CNSi, characterizing an incidence of 2.17% (7/322). Comparing groups of patients with and without CNSi, we observed that the lactate dehydrogenase (LDH), number of extranodal sites, IPI, kidney/adrenal and absence of complete response were statistically different. The CNS-IPI model stratified patients in a three-risk group model as low-, intermediate- and high-risk. In our cohort, using the same stratification, we obtained an equivalent the 2-year rate of CNS relapse of 0.0%, 0.8% and 13.8%, respectively. Conclusion: Our study reinforces the reproducibility of the CNS-IPI, specifically apart from clinical trials, and suggests the CNS-IPI score as a tool to guide therapy.

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)

Adolescence Pseudolymphoma / Pseudolinfoma na adolescência

Objective: report an uncommon case of cutaneous pseudolymphoma in teenage years, undiagnosed for approximately 8 years old. Methodology: data were taken from medical records, patient interviews, photographic records of the injuries, diagnostic methods, and literature review. The paper was subjected and approved by the Research Ethics Committee (REC), under the number 4.952.193, authorized by the patient and their legal sponsor. Final Conclusions: the related case shows the importance of reliable and differential diagnoses since the patient carried the injury through approximately eight years without getting any diagnosis and/or treatment. Furthermore, the unusual age and the location of the injuries make the information presented here fundamental to helping other professionals and contributing to the Public Health System (AU).

Objetivo: Relatar um caso incomum de Pseudolinfoma Cutâneo na adolescência, não diagnosticado por aproximadamente oito anos. Método: As informações foram obtidas pela revisão do prontuário, entrevista com o paciente, registro fotográfico das lesões e dos métodos diagnósticos e revisão de literatura. O trabalho foi submetido e aprovado pelo Comitê de Ética em Pesquisa (CEP), número de aprovação 4.952.193, com autorização do paciente e seus responsáveis legais. Considerações Finais: o caso relatado evidencia a importância de um diagnóstico fidedigno e dos diagnósticos diferenciais, uma vez que a paciente apresentou a lesão por aproximadamente oito anos, sem receber nenhum diagnóstico e/ou tratamento. Além disso, não só a faixa etária é incomum, mas também a localização da lesão e por esta razão, as informações são fundamentais para auxiliar outros profissionais, com benefício deste estudo para o Sistema de Saúde (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 difuso de células B grande como linfoma primario de ovario. Presentación de caso / Diffuse large B-cell non-Hodgkin's lymphoma as primary ovarian lymphoma. Case presentation

Fundamento: Los linfomas primarios de ovario son poco frecuentes; el 1 % de estos se presenta en ovario y el 1.5 % de los tumores malignos de ovario son linfomas. Los tipos histológicos más frecuentes es el linfoma no Hodgkin difuso de células B grande y el BurKitt; el tratamiento consiste en cirugía combinada con quimioterapia. Objetivo: Reportar un caso de un linfoma no Hodgkin difuso de células B grande primario de ovario. Presentación de caso: Se presentó el caso de una paciente de 39 años de edad, con antecedentes patológicos personales de salud; la cual fue al cuerpo de guardia de ginecología por presentar dolor abdominal difuso que no se aliviaba con analgésicos. En la exploración física presentaba dolor a la palpación superficial y profunda en hipocondrio y fosa ilíaca derecha con masa tumoral palpable. Ecografía hacia proyección anexial derecha se observó una imagen de baja ecogenicidad y en la laparoscopia de urgencia se concluyó como una formación de aspecto tumoral que parecía corresponderse con ovario derecho. Se le realizó una histerectomía con doble anexectomía. El diagnóstico anatomopatológico fue un linfoma no Hodgkin primario de ovario. Conclusiones: La paciente del caso presentado tuvo una clínica oligosintomática y la confirmación de la enfermedad fue a partir de una muestra quirúrgica, lo que expresa que el diagnóstico del linfoma no Hodgkin de células B es difícil y aunque es poco frecuente siempre se debe tener en cuenta en el diagnóstico diferencial de las tumoraciones unilaterales de ovario.

Background: Primary ovarian lymphomas are uncommon, 1% of these malignancies occur in the ovary, and 1.5% of all ovarian malignancies are lymphomas. The most common histologic types are diffuse large B-cell non-Hodgkin's lymphoma and BurKitt's lymphoma; treatment consists of surgery combined with chemotherapy. Objective: To report a case of primary ovarian diffuse large B-cell non-Hodgkin lymphoma. Case presentation: A 39-year-old female case is presented, with a personal pathological history; she went to the gynecology emergency service because she presented diffuse abdominal pain that was not relieved by analgesics. Physical examination revealed superficial and deep pain on palpation in the hypochondrium and right illiac fossa with a palpable tumor mass. Right adnexal ultrasound showed an image of low echogenicity and at the emergency laparoscopy, it was diagnosed as a tumor-like formation that appeared to correspond to the right ovary. She underwent a hysterectomy with double adnexectomy. The anatomopathologic diagnosis was primary ovarian non-Hodgkin's lymphoma. Conclusions: The patient in the presented case had an oligosymptomatic clinical presentation. Confirmation of the disease was obtained from a surgical sample, which means that B-cell non-Hodgkin's lymphoma is difficult to diagnose and although it is uncommon, it should always be considered in the differential diagnosis of unilateral ovarian tumors.

Children and adolescents with non-Hodgkin lymphoma in Brazil: A national survey identifies the challenges encountered and documents the diversity of care

ABSTRACT Introduction: Brazil has many inequities in the healthcare provided nationwide. Therefore, in order to access challenges in treatment, available resources and current practices, to identify barriers in delivering a good quality of care among Brazilian centers treating children and adolescents with Non-Hodgkin Lymphoma (NHL) and to generate a future prospective guideline, a customized online survey was distributed to pediatric hematologists and oncologists across the country. Results: A total of 97 surveys were completed (35% response rate), from 47 cities in all Brazilian regions and 79 units of care, with a median of 1 answer by the center (range 1 - 5). Most respondents work at an institution supported exclusively by public/philanthropic resources (58%), with an average of 5 to 9 new cases/year (49%), and 41% have 4 to 6 oncologists/centers. Additionally, 22% have no easy access to the intensive care unit, 26% have no access to Rasburicase, 28% have no access to Rituximabe as front-line therapy and 41% have unreliable methotrexate monitoring levels. Those differences cannot be explained thoroughly by regional wealth variances, nor by the financing model. Regarding the pathology service, 70% consider having reasonable quality assistance, but the timeframe to deliver diagnosis is satisfactory to 46%. There is no uniform management of care, with the current guideline from the Sociedade Brasileira de Oncologia Pediátrica being adopted by 54 to 59%, depending on the NHL subtype. Conclusion: This study provides insights into the heterogeneity of care among Brazilian centers. Recognizing those diversities will support the design of effective strategies and collaboration nationwide.

Aspectos clínicos do câncer durante o período gestacional: desafios diagnósticos e terapêuticos / Clinical aspects of cancer during the gestational period: diagnostic and therapeutic challenges

O câncer é uma das maiores causas de morte em mulheres na idade reprodutiva e ocorre em aproximadamente 0,05% a 0,1% das gestações. Os cânceres ginecológicos, de mama, tireoide e hematológicos são os mais comuns na gravidez. O obstetra é o principal médico para investigar sintomas que podem estar relacionados à malignidade. O diagnóstico pode ser dificultado devido à sobreposição de sintomas da gravidez, como náusea, vômitos, aumento do útero e das mamas, dor abdominal, além da limitação para uso de exames de imagem e alterações comuns em exames laboratoriais. O risco e o benefício do diagnóstico e o tratamento para o bem-estar materno e fetal devem ser avaliados com cuidado pelos profissionais envolvidos. Este artigo tem como objetivo realizar uma revisão sobre quando suspeitar e como investigar os principais cânceres na gestação.(AU)

Cancer is the major cause of death in women on reproductive age and occurs in approximately 0.05% to 0.1% of pregnancies. Gynecological, breast, thyroid and hema- tological cancers are the most common in pregnancy. The obstetrician is the primary physician to investigate symptoms that may be related to malignancy. The diagnosis can be difficult due to the overlap of pregnancy symptoms, such as nausea, vomiting, enlargement of the uterus and breasts, abdominal pain, in addition to the limitation for the use of imaging tests and common changes in laboratory tests. The risk and be- nefit of diagnosis and treatment for maternal and fetal well-being should be carefully assessed by the professionals involved. This article aims to conduct a review on when to suspect and how to investigate the main cancers in pregnancy.(AU)

Peritoneal lymphomatosis. A case report

ABSTRACT Introduction: Involvement of the peritoneum occurs very rarely and is exceptional as an exclusive extranodal presentation of lymphomas. In most cases lymphomas associated with this rare entity are high-grade ones. PL secondary to high-grade nodal lymphoma is more frequent than primary peritoneal lymphoma, and there are only a few cases of the latter described in the literature. Description of the case: We present the case of a patient with constitutional syndrome and imaging findings suggestive of peritoneal carcinomatosis who was finally diagnosed with a Diffuse Large B-cell Lymphoma (DLBCL) by an ultrasound-guided core needle biopsy (CNB) of peritoneum. The patient received one polychemotherapy cycle; however tumor lysis syndrome occurred with death of the patient in the following days. This case tries to show the existence of a PL without other radiological findings of lymphoma, a fact that is very exceptionally described in the literature. Discussion: The differential diagnosis between PL and others peritoneum diseases such as peritoneal carcinomatosis, malignant primary peritoneal mesotheliomas, tuberculous peritonitis, sarcomatosis, diffuse peritoneal leiomyomatosis or benign splenosis, constitutes a major problem in imaging techniques. An exhaustive analysis of the radiological characteristics as well as a clinical-analytical context allows the differential diagnosis against peritoneal carcinomatosis and the rest of the entities previously referred although the final diagnosis will always be a biopsy. Conclusion: PL usually manifests as an aggressive histological subtype of high-grade lymphomas leading to a rapid progression and deterioration of the patient. It is crucial for the radiologist and the clinician to be aware of this rare entity providing the earliest possible diagnosis and optimal treatment to prolong the patient's life.

Recaída de linfoma folicular de alto grado en tiroides / Relapse of high grade follicular lymphoma in thyroid

El linfoma folicular de tiroides es una entidad rara que representa menos del 2% de los linfomas extranodales y cuyo principal factor de riesgo es la tiroiditis de Hashimoto. Suele afectar con mayor predisposición a pacientes adultos mayores, en los que con frecuencia se presenta como una masa de crecimiento rápido con compromiso de las estructuras adyacentes en el cuello. Sin embargo, dada su rara incidencia, esta patología debe ser sospechada por el clínico para un diagnóstico temprano oportuno. Aunque en la actualidad no existen guías de manejo estandarizadas para esta entidad, acostumbra tener buena respuesta a radioterapia y quimioterapia. El presente caso describe la historia de un paciente con una recaída de linfoma no Hodgkin de tipo folicular de alto grado localizado en tiroides que se presentó a urgencias con disfonía, parálisis bilateral de cuerdas vocales, cambio en el tono de la voz y disfagia, con el antecedente de un linfoma folicular de alto grado en localización retroperitoneal y en remisión luego de 8 ciclos de quimioterapia con rituximab, ciclofosfamida, doxorubicina, vincristina y prednisona, el cual presentó citorreducción luego del tratamiento con quimioterapia con el protocolo de rituximab, dexametasona, citarabina y cisplatino.

Follicular thyroid lymphoma is a rare entity that represents less than 2% of extranodal lymphomas; and whose main risk factor is Hashimoto's thyroiditis. This tends to affect older adult patients with greater predisposition, where it usually presents as a rapidly growing mass with compromise of the adjacent structures in the neck. However, given its rare incidence, this pathology should be suspected by the clinician for an early diagnosis. Although there are currently no standardized management guidelines for this entity, it usually responds well to radiotherapy and chemotherapy. The present case describes the history of a patient with a relapse of high-grade follicular-type NHL located in the thyroid who presented to the emergency room with dysphonia, bilateral paralysis of the vocal cords, change in tone of voice and dysphagia, with the history of high-grade follicular lymphoma in retroperitoneal location in remission after 8 cycles of chemotherapy with Rituximab, Cyclophosphamide, Doxorubicin, Vincristine and Prednisone, which presented cytoreduction after treatment with chemotherapy with the protocol Rituximab, Dexamethasone, Cytarabine and Cisplatin.

Linfoma del tejido linfoide asociado a las mucosas en paciente con tiroiditis de Hashimoto / Mucosa-associated lymphoid tissue lymphoma in a patient with Hashimoto's thyroiditis

RESUMEN El linfoma del tejido linfoide asociado a las mucosas es una variedad poco común y recientemente descubierta del linfoma no Hodgkin. Suele presentarse en la sexta década de la vida, con un predominio del sexo femenino y en sitios como el tracto digestivo, pulmón, riñón, hígado, piel, y solo en el 2 % de los casos, en la glándula tiroides, donde en muchas ocasiones se asocia a la tiroiditis autoinmune de Hashimoto. Su evolución es favorable cuando se diagnostica en estadios iniciales de la enfermedad. Se presenta una paciente de 22 años, con una historia de trastornos endocrinos, perceptibles desde la adolescencia, a quien se le diagnosticó una tiroiditis de Hashimoto, sobre la que subyacía un linfoma del tejido linfoide asociado a las mucosas, y que evolucionó satisfactoriamente luego del tratamiento quirúrgico.

ABSTRACT Mucosa-associated lymphoid tissue lymphoma is a rare and recently discovered variant of non-Hodgkin's lymphoma. It usually occurs in the sixth decade of life, with a predominance of females and may be observed in sites such as the digestive tract, lung, kidney, liver, skin, and only in 2% of cases, in the thyroid gland, where in many occasions it is associated with Hashimoto's autoimmune thyroiditis. Its evolution is favorable when it is diagnosed in the initial stages of the disease. We present a 22-year-old female patient with a history of perceptible endocrine disorders since adolescence, who was diagnosed with Hashimoto's thyroiditis, underlying mucosa-associated lymphoid tissue lymphoma, and who evolved satisfactorily after surgical treatment.

Asociación del inmunofenotipo linfocitario de sangre periférica con la presencia de linfomas no Hodgkin / Association of peripheral blood lymphoid immunophenotype with presence of non-Hodgkin lymphomas

Introducción: Los cambios en el inmunofenotipo de los linfocitos en los pacientes con linfoma no Hodgkin están asociados con el pronóstico y las respuestas terapéuticas. Sin embargo, no se ha establecido sistemáticamente la asociación con la enfermedad y por tanto su contribución al diagnóstico. Objetivo: Evaluar la asociación del inmunofenotipo linfocitario en sangre periférica con la presencia del linfoma no Hodgkin. Métodos: Se analizaron 31 muestras de sangre periférica de pacientes con diagnóstico confirmado de linfoma no Hodgkin y de 68 individuos sanos como controles, durante el período de 2018 a 2020. Se empleó la citometría de flujo multiparamétrica para el inmunofenotipado. Se calculó el área bajo la curva y el índice de Youden para establecer puntos de corte en los porcentajes linfocitarios. La asociación de los cambios inmunofenotípicos con el linfoma no Hodgkin, se realizó mediante cálculos de Odd ratio. Resultados: El aumento de linfocitos TCD8+ y NKCD56opaco se asoció significativamente con la presencia de linfoma no Hodgkin (OR= 3,4 y 2,9; respectivamente). Por el contrario, la disminución de linfocitos TCD4+, T doble positivo, T doble negativo y NKCD56brillante también se asoció con la existencia de linfoma no Hodgkin (OR= 23,0; 10,7; 6,9 y 15,8; respectivamente). Además, la disminución del índice CD4/CD8 también fue asociada con la enfermedad. Conclusiones: Los cambios encontrados en los inmunofenotipos linfocitarios se asociaron de forma significativa con la presencia del linfoma no Hodgkin, lo cual representa una expresión sistémica de la enfermedad y sugiere su valor diagnóstico(AU)

Introduction: Lymphocyte immunophenotype changes in non-Hodgkin lymphoma patients are associated with prognosis and therapeutic responses. However, its association with the disease has not been systematically established. Therefor its contribution to the diagnosis process. Objective: To assess the association of lymphocyte immunophenotype in peripheral blood with the presence of non-Hodgkin lymphoma. Methods: 31 peripheral blood samples were analyzed from patients with a confirmed diagnosis of non-Hodgkin lymphoma and from 68 healthy individuals as controls, during the period 2018 to 2020. Multiparametric flow cytometry was used for immunophenotyping. The area under the curve and the Youden index were calculated to establish cut-off points in lymphocyte percentages. The association of immunophenotypic changes with non-Hodgkin's lymphoma was made using Odd ratio calculations. Results: The increase in TCD8+ and NKCD56dim lymphocytes from peripheral blood was significantly associated with the presence of non-Hodgkin lymphoma (OR= 3.4 and 2.9, respectively). Oppositely, the decrease in TCD4+, double positive T, double negative T and NKCD56bright lymphocytes was associated with the existence of non-Hodgkin lymphoma (OR= 23.0, 10.7, 6.9 and 15.8, respectively). Therefore, the decrease in the CD4/CD8 rate was also associated with the disease. Conclusion: The changes found in these lymphocytic immunophenotypes were significantly associated with the presence of non-Hodgkin lymphoma, which represents a systemic expression of the disease and suggests its diagnostic value(AU)

Linfoma ocular primario vs celulitis orbitaria en tiempos de COVID-19 / Primary ocular lymphoma vs orbital cellulitis in times of COVID-19

Los tumores sólidos neoplásicos de tejido linfoide se caracterizan por proliferación acelerada de la porción linforreticular del sistema retículo endotelial, su incidencia mundial es de 4/100,000 prevalente en féminas entre 50-70 años, de etiología desconocida, asociado a helicobacter pylori, epstein barr y VIH. Clínicamente están presentes los síntomas B: fiebre, sudoraciones nocturnas, pérdida de peso, prurito y astenia. ausentes en el caso que nos ocupa de una paciente femenina de 46 años de edad que acudió a consulta de oftalmología por aumento de volumen a nivel de anejos de ojo izquierdo, proptosis indolora y disminución de la visión, con diagnóstico inicial de celulitis orbitaria tratada ambulatoriamente sin resolución. Se administró antibioticoterapia intravenosa sin mejoría clínica, se realizaron estudios complementarios de imagen radiológica, ultrasonido, resonancia magnética con evidencia de imagen tumoral de crecimiento antero lateral, desplazamiento de globo ocular, diámetro mayor de 57,3 mm, de características isointensas heterogéneas, secuencia T1 y flair, erosión de pared interna de orbita y la biopsia excisional informó linfoma primario de anexo ocular orbitario tipo no-Hodgkin. El abordaje acucioso con estudios complementarios para descartar neoplasias orbitarias en pacientes con celulitis orbitaria o dacriocistitis de evolución tórpida es necesario en todo momento, independiente de las condiciones atípicas de pandemia por COVID-19 que dificultan su manejo(AU)

Solid neoplastic tumors of lymphoid tissue are characterized by accelerated proliferation of the lymphoreticular portion of the reticulum endothelial system, their worldwide incidence is 4/100,000 prevalent in females between 50-70 years of age, of unknown etiology, associated with helicobacter pylori, epstein barr and HIV. Clinically, symptoms B are present: fever, night sweats, weight loss, itching and asthenia, absent in the present case of a 46-year-old female patient who came to the ophthalmology office due to increased volume at the level of the appendages. of the left eye, painless proptosis and decreased vision, with an initial diagnosis of orbital cellulitis treated outpatiently without resolution. Intravenous antibiotic therapy was administered without clinical improvement, complementary radiological imaging studies, ultrasound, magnetic resonance imaging were performed with evidence of an anterolateral growth tumor image, ocular globe displacement, diameter greater than 57.3 mm, heterogeneous isointense characteristics, T1 sequence and flair, erosion of the internal wall of the orbit and the excisional biopsy reported primary non-Hodgkin type orbital annex lymphoma. A careful approach with complementary studies to rule out orbital neoplasms in patients with orbital cellulitis or dacryocystitis of torpid evolution is necessary at all times, regardless of the atypical conditions of a COVID-19 pandemic that make its management difficult(AU)

Lymphomatous Polyposis: Case Report and Literature Review / Poliposis linfomatosa: reporte de caso y revisión de la literatura

Abstract Lymphomatous polyposis (LP) is the endoscopic feature of primary gastrointestinal mantle cell lymphoma (MCL), a rare type of B-cell non-Hodgkin's lymphoma (NHL) and a typical but rare endoscopic pattern of gastrointestinal tract involvement (GIT) by nodal MCL. We present the case of a 62-year-old man with nodal MCL, with LP as a manifestation of GIT, and review the literature.

Resumen La poliposis linfomatosa (PL) es la característica endoscópica del linfoma de células del manto (LCM) gastrointestinal primario, un tipo infrecuente de linfoma no Hodgkin (LNH) de células B, así como un patrón endoscópico típico, pero infrecuente, del compromiso del tracto gastrointestinal (TGI) por LCM nodal. Presentamos el caso de un hombre de 62 años con LCM nodal, con PL como manifestación del compromiso gastrointestinal, y realizamos una revisión de la literatura.

Trabalho rural associado a cânceres linfohematopoiéticos em hospital público de referência: estudo caso-controle, Ceará, Brasil, 2019-2021 / Rural labor associated with lymphohematopoietic cancers in a public reference hospital: a case-control study, Ceará, Brazil, 2019-2021 / Trabajo rural asociado a cánceres linfohematopoyéticos en un hospital público de referencia: estudio de caso control, Ceará, Brasil, 2019-2021

Trata-se de um estudo do tipo caso-controle pareado com o objetivo de verificar a associação entre ocupações e cânceres linfohematopoiéticos em um hospital público de referência no Estado do Ceará, Brasil, durante 2019-2021. O grupo caso foi constituído por pacientes hematológicos que apresentavam mieloma múltiplo, leucemias ou linfomas não Hodgkin, acompanhados pelo serviço social de uma unidade hospitalar de transplante de medula óssea (n = 114), enquanto indivíduos comparáveis de unidade hospitalar distinta constituíram o grupo controle (n = 114), formando pares 1:1. O diagnóstico foi efetuado por equipe médica e as variáveis foram aferidas por acesso aos registros hospitalares. Comparamos os grupos em regressão logística condicional bivariada e ajustada por região de residência. Entre os resultados, destacamos que o câncer de maior prevalência no grupo caso foi o mieloma múltiplo (43,9%), seguido pelas leucemias (43%) e por linfomas não Hodgkin (13,2%). Proporções de ocupações, zona de residência, abastecimento de água e Superintendência Regional de Saúde de residência apresentaram diferença estatisticamente significante entre os grupos caso e controle. Verificamos que trabalhadores rurais possuíam maiores chances de apresentar os cânceres estudados (ORbruto = 5,00, IC95%: 1,91; 13,06 e ORajustado = 3,38, IC95%: 1,20; 9,54), enquanto trabalhadores do comércio apresentaram menores chances (ORbruto = 0,26, IC95%: 0,10; 0,70 e ORajustado = 0,30, IC95%: 0,10; 0,88). Os achados deste estudo possibilitam reflexões sobre o processo de adoecimento dos trabalhadores rurais e refletem o potencial dos serviços de saúde em contribuir com investigações sobre exposições ocupacionais.

This is a paired case-control study that sought to verify the association between professions and lymphohematopoietic cancers in a public reference hospital in the State of Ceará, Brazil, during 2019-2021. Case group consisted of hematological patients with multiple myeloma, leukemias or non-Hodgkin lymphomas, monitored by the social service of a bone marrow transplant hospital unit (n = 114), whereas comparable individuals from a different hospital unit made up the control group (n = 114), forming 1:1 pairs. Diagnosis was performed by a medical team and the variables were measured by accessing hospital records. Group comparison was conducted using bivariate conditional logistic regression and adjusted by region of residence. Results show that multiple myeloma (43.9%), leukemias (43%) and non-Hodgkin lymphomas (13.2%) were the cancer with the highest prevalence in the case group. Proportions of jobs, area of residence, water supply and Regional Superintendence of Health showed statistically significant difference between the case and control groups. Rural workers were more likely to present the cancers studied (ORraw = 5.00, 95%CI: 1.91; 13.06 and ORadjusted = 3.38, 95%CI: 1.20; 9.54), whereas trade workers had lower odds (ORraw = 0.26, 95%CI: 0.10; 0.70 and ORadjusted = 0.30, 95%CI: 0.10; 0.88). The findings allow us to reflect on the process of illness among rural workers and illustrate the potential of health services to contribute to investigations on occupational exposures.

Se trata de un estudio del tipo caso control apareado con el objetivo de verificar la asociación entre ocupaciones y cánceres linfohematopoyéticos en un hospital público de referencia en el estado de Ceará, Brasil, durante el período 2019-2021. El grupo caso estaba formado por pacientes hematológicos que presentaban mieloma múltiple, leucemias o linfomas no Hodgkin, bajo seguimiento por parte del servicio social de una unidad hospitalaria de trasplante de médula ósea (n = 114), mientras que individuos comparables de otra unidad hospitalaria constituyeron el grupo control (n = 114), formando pares 1:1. El diagnóstico fue realizado por un equipo médico, y las variables se midieron accediendo a los registros hospitalarios. Comparamos los grupos en regresión logística condicional bivariada y ajustada por región de residencia. Entre los resultados, destacamos que el cáncer de mayor prevalencia en el grupo caso fue el mieloma múltiple (43,9%), seguido de las leucemias (43%) y los linfomas no Hodgkin (13,2%). Las proporciones de ocupaciones, zona de residencia, abastecimiento de agua y Superintendencia Regional de Salud de residencia presentaron diferencia estadísticamente significativa entre los grupos caso y control. Constatamos que los trabajadores rurales tenían más probabilidades de padecer los cánceres estudiados (ORcrudo = 5,00, IC95%: 1,91; 13,06 y ORajustado = 3,38, IC95%: 1,20; 9,54), mientras que los trabajadores del comercio presentaron menos posibilidades (ORcrudo = 0,26, IC95%: 0,10; 0,70 y ORajustado = 0,30, IC95%: 0,10; 0,88). Los hallazgos de este estudio permiten reflexiones sobre el proceso de enfermedad de los trabajadores rurales y reflejan el potencial de los servicios de salud para contribuir a las investigaciones sobre exposiciones ocupacionales.

Linfoma cardíaco primário: o papel da multimodalidade de imagem na abordagem diagnóstica / Primary cardiac lymphoma: role of imaging multimodality in diagnosis

Massas intracardíacas constituem um desafio diagnóstico, já que os sintomas são comuns a patologias cardiovasculares ou não. Métodos ­ invasivos ou não ­ possibilitam o diagnóstico diferencial e a confirmação histológica, propiciando tratamento adequado. Para melhor compreender a importância da multimodalidade em imagem e a abordagem nos tumores cardíacos , relatamos o caso de um lifoma cardíaco primário, em que a abordagem multidisciplinar permitiu o rápido diagnóstico e seu tratamento, inclusive das intercorrências, com resposta inicial promissora, a despeito da evolução fatal durante a pandemia por SARS-CoV-2.(AU)

Intracardiac masses are a diagnostic challenge since their symptoms can be common to cardiovascular pathologies. Some methods, whether invasive or not, enable differential diagnosis, histological confirmation, and adequate treatment. To better understand the importance of imaging multimodality and the approach to managing cardiac tumors, we investigated a case of a primary cardiac lymphoma in which the multidisciplinary approach allowed rapid diagnosis and treatment, including of intercurrences, with a promising initial response despite fatal progression due to severe acute respiratory syndrome coronavirus 2 infection. (AU)

¿Cuándo deben operarse los quistes de retención en los senos paranasales? Revisión de la literatura / When should retention cysts in paranasal sinuses be operated? Literature review

Introducción: Los quistes de retención mucoso de los senos paranasales son un hallazgo imagenológico incidental muy frecuente. Usualmente comprometen el seno maxilar. Metodología: Se realizó una amplia búsqueda de la literatura, sobre quistes de retención mucoso nasosinusales, en enero de 2021 en múltiples bases de datos. Se seleccionaron 21 artículos sobre el tema para la realización de la revisión. Discusión: En la mayoría de los casos el tratamiento de los quistes de retención mucoso nasosinusales debe ser expectante debido a su tendencia a la involución y curso asintomático. Sin embargo, en la práctica clínica se observa con frecuencia un manejo quirúrgico sin una indicación clara. Conclusiones: Las indicaciones quirúrgicas de los quistes de retención mucoso son: obstrucción del ostium del seno paranasal, compromiso del complejo ostiomeatal o compromiso del nervio infraorbitario con síntomas secundarios. Es importante que el cirujano reconozca la naturaleza y comportamiento de esta patología para evitar procedimientos quirúrgicos innecesarios.

Introduction: Mucous retention cysts of the paranasal sinuses are a very common incidental imaging finding. They usually involve the maxillary sinus. Methodology: An extensive literature search on nasosinusal mucosal retention cysts was conducted in January 2021 through multiple databases. 21 articles on the subject were included as evidence for this paper. Discussion: In most cases, treatment should be expectant due to its tendency to involution and asymptomatic course. However, in clinical practice, surgical management is frequently observed without a clear indication. Conclusions: Surgical indications for mucosal retention cysts are: obstruction of the sinus ostium, involvement of the ostiomeatal complex, or involvement of the infraorbital nerve with secondary symptoms. It is important for the surgeon to recognize the nature and behavior of this pathology in order to avoid unnecessary surgical procedures.

Proteinosis alveolar pulmonar: reporte de caso en paciente con linfoma no hodgkin mesentérico / Pulmonary alveolar proteinosis: report of a case in patient with history of mesenteric non-hodgkin lymphoma

Objetivos: Exponer en base a un caso clínico una revisión de literatura reciente sobre Proteinosis alveolar pulmonar (PAP). Presentación del caso: Revisión de ficha clínica electrónica de paciente de sexo masculino de 76 años con antecedente de linfoma no Hodgkin (LNH) mesentérico, estirpe B de tipo folicular, quien acude en forma reiterada a servicios de urgencia por cuadro de dos meses de evolución de fiebre, compromiso del estado general y tos. Al examen físico destaca crépitos en hemitórax derecho. Se realizó Tomografía computada (TC) de tórax que mostró opacidades pulmonares en vidrio esmerilado periféricas, con engrosamiento septal liso y algunas bandas retráctiles subpleurales. Se manejó ambulatoriamente con Azitromicina por una semana. Sin respuesta, evoluciona con baja de peso y diaforesis nocturna. Nueva TC de tórax en enero 2021, muestra nuevos focos de "empedrado" periféricos extensos, descrito como "crazy paving", focos de vidrio esmerilado difusos extensos, sin condensación y con resolución de bandas retráctiles. Estudio infeccioso negativo. Se realiza lavado broncoalveolar (LBA) con estudio histológico de líquido que muestra proceso inflamatorio crónico con abundantes macrófagos y material proteináceo. Discusión: Tras el descarte de patología infecciosa, se orientó el estudio hacia otras causas de enfermedad parenquimatosa pulmonar. Así, resulta fundamental la descripción correcta del patrón imagenológico tomográfico y el LBA que resultaron compatibles con PAP. Conclusión: La PAP es una patología infrecuente, pero una historia clínica adecuada, el planteamiento de diagnósticos diferenciales de neumonía de lenta resolución, asociado el reconocimiento del patrón radiológico característico y el estudio histológico con LBA permiten realizar un diagnóstico certero, con gran implicancia terapéutica.

Objective: To present a review of recent literature on pulmonary alveolar proteinosis (PAP) based on a clinical case. Presentation of the case: Review of electronic clinical record of a 76 years-old masculine patient with history of mesenteric Non-Hodgkin Lymphoma (NHL) follicular-type lineage B, who repeatedly attended the emergency services due to a two-month history of symptoms of fever, compromised general condition and cough. Physical examination revealed crepitus in the right hemithorax. Chest computed tomography (CT) was performed, which showed peripheral ground-glass pulmonary opacities, with smooth septal thickening and some subpleural retractile bands. He was managed on an outpatient basis with Azithromycin for one week. No response; evolves with weight loss and night diaphoresis. New chest CT in January 2021, shows new extensive peripheral "cobblestone" foci, described as "crazy paving", extensive diffuse ground glass foci, without condensation and with resolution of retractile bands. Negative infectious study. Bronchoalveolar lavage (BAL) was performed with a histological study of the fluid showing a chronic inflammatory process with abundant macrophages and proteinaceous material. Discussion: After ruling out infectious pathology, the study was oriented towards other causes of pulmonary parenchymal disease. Thus, the correct description of the tomographic imaging pattern and the BAL that were compatible with PAP are essential. Conclusion: PAP is an infrequent pathology, but an adequate clinical history, the approach to differential diagnoses of slowly resolving pneumonia, associated with the recognition of the characteristic radiological pattern and the histological study with BAL allow an accurate diagnosis to be made, with great therapeutic implications.

Linfoma con morfología en anillo de sello: patología con distintivos diagnósticos diferenciales / Lymphoma with signet ring morphology: pathology with distinctive differential diagnoses

Introducción: el linfoma con células en anillo de sello es una entidad poco frecuente y simuladora de neoplasias epiteliales, sarcomas y condiciones reactivas de histiocitos. Representa una variante morfológica de distintos linfomas no Hodgkin, por lo que su diagnóstico puede representar un desafío y debe ser considerado al realizar estudios complementarios. Objetivo: mostrar un caso con una morfología muy poco frecuente y recalcar la importancia de conocer esta entidad para no cometer errores en su diagnóstico. Caso clínico: se documenta el caso de un hombre de 67 años que desarrolló crecimientos ganglionares en axila derecha, cuello, ingle derecha, y región submandibular, con aparente afección en pulmones y bazo, que fue diagnosticado como linfoma B difuso de células grandes con morfología en anillo de sello, originado en el centro germinal. Se realizó estudio de microscopía electrónica de transmisión para una mejor caracterización de la morfología. Desafortunadamente el paciente no regresó a consulta de seguimiento, por lo que no inició tratamiento, falleció a los 6 meses posteriores al diagnóstico. Conclusiones: el linfoma con fenotipo en anillo de sello es poco frecuente, y puede presentarse en cualquier tipo de linfomas no Hodgkin; sin embargo, esta morfología es más comúnmente asociada a carcinomas y, en menor frecuencia, a sarcomas, melanomas o histiocitos reactivos, por lo que el considerar esta entidad junto con el uso adecuado de estudios complementarios es de gran importancia para su adecuado diagnóstico.

Background: Signet-ring cell lymphoma is a rare entity that simulates epithelial neoplasms, sarcomas and reactive histiocytes conditions. It represents a morphological variant of non-Hodgkin's lymphomas, its diagnosis can represent a challenge, therefore it should be considered in complementary studies. Objective: The aim of this work is to show a case with a very rare morphology and to emphasize the importance of awareness this entity and avoid mistakes in its diagnosis. Clinical case: We present a case of a 67-year-old man, who developed lymph node growths in the right armpit, neck, right groin, and submandibular region, with apparent involvement of the lungs and spleen; was diagnosed as diffuse large B cell lymphoma with signet-ring morphology, originated in the germinal center. Transmission electron microscopy study was carried out for a more precise characterization of the morphology. Unfortunately, the patient did not return for a follow-up consultation, so he did not start treatment and died 6 months after diagnosis. Conclusions: Lymphoma with the signet-ring phenotype is rare, and can occur in any type of non-Hodgkin lymphoma; however, this morphology is more commonly associated with carcinomas and, less frequently, with sarcomas, melanomas or reactive histiocytes conditions, therefore should be considered this entity together with the appropriate use of complementary studies for proper diagnosis.