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

Prise en charge des hémopathies malignes au centre national d'oncologie médicale et de radiothérapie Alassane Ouattara d'Abidjan / Managem ent of hem atologic neoplasm sat the Centre National d'Oncologie Médicale et de Radiothérapie Alassane Ouattara ofAbidjan

Introduction. Les hémopathies malignes sont des proliférations anormales et anarchiques de cellules hématopoïétiques à point de départ médullaire ou périphérique. Notre étude qui avait pour objectif de faire le bilan de la prise en charge des hémopathies malignes au Centre national d'oncologie médical et de radiothérapie Alassane Ouattara. Méthodes. Il s'agissait d'une étude rétrospective descriptive d'une durée de 2 ans 3 mois allant du 1er janvier 2018 au 31 Mars 2020 portant sur 80 dossiers de malades porteurs d'hémopathies malignes et prise en charge dans le centre. Résultats. Notre étude a permis de recenser 2,2% d'hémopathies malignes sur les 3650 cas de pathologies cancéreuses recensées au CNRAO, soit une incidence 26,66 cas/an avec un sex-ratio 1,2. Les syndromes lymphoprolifératifs (SLP) sont les plus fréquents (96,25 %), sous trois principales formes: lymphomes malins non hodgkiniens (LMNH) non Burkitt (51,25%), leucémie myéloïde chronique (20%) et le myélome multiple (16,25%). Les LMNH représentent 51,25% des HM recensées avec 41,46 % de lymphomes de haut grade de malignité. La chimiothérapie était de mise chez tous nos patients. Ainsi sur 41 cas de LMNH, seulement 12 (29,27%) ont bénéficié du protocole R-CHOP. On notait 46,34% de réponse complète. Dans notre étude, le LH représentait 8,75% il était traité à 71,43% avec le protocole ABVD avec une réponse complète chez 6 patients. 37,5% des patients porteurs de leucémie myéloïde chronique ont reçu le Rituximab; ils ont été traités par les protocoles COP (31,25%), CHOP (31,25%), RCVP (12,5%) et R-CHOP (25%). La réponse thérapeutique était complète à 68,75%. Le protocole utilisé dans le traitement du myélome multiple a été le VMCD-REV à 76,92% avec pour réponse thérapeutique complète chez 6 patients, 3 réponses partielles et 4 en cours de traitement. Conclusion. Les SLP qui sont les plus fréquents des HM avec trois principales formes: LMNH non Burkitt, leucémie myéloïde chronique et myélome multiple. Nous avons cependant des difficultés quant à la mise en route de la chimiothérapie.

Introduction. Hematologic neoplasms are abnormal and anarchic proliferations of hematopoietic cells with a medullary or peripheral starting point. Our study aimed to report the management of hematological malignancies at the Centre National d'Oncologie Médicale et de Radiothérapie Alassane Ouattara (CNRAO). Methods. This was a descriptive retrospective study lasting 2 years 3 months from January 1st, 2018 to March 31st, 2020 concerning 80 patients with hematologic neoplasms who were managed in the CNRAO. Results. Hematologic neoplasms represented 2.2% of cancers (80/3650) at CNRAO, giving an annual incidence of 26.66 cases. The sex ratio was 1.2. Lymphoproliferative syndromes were the most common subgroups (96.25%). These were mainly non-Burkitt non Hodgkin lymphoma (51.25%), high grade lymphomas (41.46%), chronic lymphocytic lymphoma (20%) and multiple myeloma (16.25%). Chemotherapy was administered to all patients. Among 41 cases of non-Hodgkin lymphoma, 12 (29.27%) benefited from the R-CHOP protocol and full response was observed in 46.34% of them. We found 7 patients with Hodgkin lymphoma (8.75%) and the ABVD protocol was used for 6 cases (71.43%). Six out of these seven patients were in complete response. Among the 16 patients with chronic lymphocytic leukemia, 6 (37.5%) received Rituximab. The distribution of the patients with chronic lymphocytic leukemia was as follows: COP 31.25%, CHOP 31.25%, RCVP 12.5% and R-CHOP 25% and 68.75% had full response. The most common treatment protocol for multiple myeloma was VMCD-REV (76.92%). Six patients had complete response, 3 had partial response and 4 were in the course of treatment. Conclusion. In our practice, hematologic neoplasms are mainly lymphoproliferative syndromes and the most common varieties are non-Burkitt non Hodgkin lymphoma, high grade lymphomas, chronic lymphocytic lymphoma and multiple myeloma. We have difficulties in getting chemotherapy started.

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.

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)

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.

Analysis of Agranulocytosis Time and Its Influencing Factors in Patients with Hematological Malignancies Treated with rhIL-11 combined rhG-CSF / 中国实验血液学杂志

OBJECTIVE@#To explore the intervention effect of recombinant human interleukin-11 (rhIL-11) and recombinant human granulocyte-colony stimulating factor (rhG-CSF) on the duration and severity of agranulocytosis in patients with hematological malignancies after chemotherapy, and to analyze the influencing factors.@*METHODS@#The data of hematological malignancy patients treated with rhIL-11 and rhG-CSF after chemotherapy in the hematology department of The First Hospital of Lanzhou University from July 2017 to July 2020 were collected retrospectively. The duration and differences of agranulocytosis in differeent groups were compared by univariate analysis, and the influencing factors of agranulocytosis duration were further analyzed by multiple regression analysis.@*RESULTS@#The duration of agranulocytosis in 97 patients was 6.47±2.93 days. The results of univariate analysis showed that there were no statistical differences in the duration of agranulocytosis among patients with different sex, age, height, weight, body surface area, body mass index (BMI), dose of rhG-CSF, dose of rhIL-11, spontaneous bleeding after administration of rhG-CSF and rhIL-11, and the duration of agranulocytosis in patients with different red blood cell count (RBC), hemoglobin(HGB) level, platelet count (PLT) and absolute neutrophil count (ANC), before administration of rhG-CSF and rhIL-11. There were significant differences in agranulocytosis time among patients with different disease types, chemotherapy cycle, fever after rhG-CSF and rhIL-11 administration, and different white blood cell count (WBC) baseline level before rhG-CSF and rhIL-11 administration (P＜0.05). Compared with patients with acute lymphoblastic leukemia (ALL) and non-Hodgkin lymphoma (NHL), patients with acute myeloid leukemia (AML) had the longest duration of agranulocytosis, which was 7.07±3.05 d. Compared with patients with chemotherapy cycles of 4－6 and ≥7, patients with total chemotherapy cycle of 1－3 had the shortest duration of agranulocytosis, which was 5.25±2.48 d. Compared with patients without fever, patients with fever within 1 day after administration of cytokines and patients with fever within 2-5 days after administration of cytokines, the duration of agranulocytosis was the longest in patients with fever 6 days after administration of cytokines, which was 8.85±2.85 d. Compared with patients with WBC baseline <1.0×109/L, (1.0－1.9)×109/L and (2.0－3.9)×109/L, patients with WBC baseline ≥4.0×109/L had the shortest duration of agranulocytosis, which was 4.50±2.56 d. Multiple linear regression analysis showed that chemotherapy cycle, different fever after administration of rhG-CSF and rhIL-11, diagnosis of ALL and NHL, and WBC baseline level before administration of rhG-CSF and rhIL-11 were the influencing factors of the duration of agranulocytosis (P＜0.001).@*CONCLUSION@#The risk of prolonged agranulocytosis is higher in patients diagnosed with AML, with more chemotherapy cycles, lower WBC baseline before cytokines administration and fever later after cytokines administration, which should be paid more attention to.

Correlation and Influencing Factors of SUVmax and Ki-67 in Non-Hodgkin Lymphoma / 中国实验血液学杂志

OBJECTIVE@#To investigate the influencing factors of maximum standard uptake value (SUVmax) of 18F-fluorodeoxyglucose (18F-FDG) positron emission tomography/computed tomography (PET/CT) and nuclear antigen Ki-67 in non-Hodgkin lymphoma (NHL) and their correlation.@*METHODS@#The relationship between SUVmax, Ki-67 and gender, age, maximum lesion diameter, extranodal involvement, superficial and deep lymph node involvement, malignancy, B symptoms, clinical stage, lactate dehydrogenase (LDH) and international prognostic index (IPI) scores and their correlation were reviewed.@*RESULTS@#Among 185 NHL patients, 99 cases were aggressive B-cell NHL, 43 cases were indolent B-cell NHL, and 43 cases were T-cell NHL, respectively. Obviously, the SUVmax and Ki-67 of aggressive B-cell NHL were higher than those of indolent B-cell NHL and T-cell NHL (P<0.05), while indolent B-cell NHL were lower than those of T-cell NHL (P<0.05). SUVmax and Ki-67 were closely related to maximum lesion diameter, extranodal involvement, malignancy, LDH, and IPI scores (P<0.05). SUVmax was positively correlated with Ki-67 expression (r=0.615). According to the analysis of receiver operating characteristic (ROC), the results showed that the SUVmax and Ki-67 could reflect the aggressiveness of NHL accurately, with an AUC of 0.871 and 0.968.@*CONCLUSION@#SUVmax and Ki-67 are not affected by age, sex, B symptoms, clinical stage and so on, and are relatively objective quantitative parameters. SUVmax is positively correlated with Ki-67 expression in NHL. SUVmax and Ki-67 have certain value in clinical diagnosis of malignant degree of NHL.

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.

Incidence des cancers chez les personnes vivant avec le VIH suivies à Casablanca / Incidence of cancer among people living with HIV at Casablanca

Objectif. Décrireles aspects épidémiologiques des cancers chez les personnes vivant avec le VIH à Casablanca. Patients et méthodes. Il s'agissait d'une étude rétrospective sur 13 ans dans le service des maladies infectieuses du CHU Ibn Rochd de Casablanca. Ont été inclus tous les patients infectés par le VIH et présentant un cancer documenté histologiquement. Les données ont été colligées à partir du Nadis. L'analyse des données a été faite sur le logiciel épi info. Une valeur de p< 0,05 était considérée significative. Résultats. Nous avons inclus 139 patients qui présentaient des pathologies cancéreuses soit un taux d'incidence de 3,7patients pour 1000 patients année. L'âge moyen était de 37ans. Le sexe ratio était de 1,72 (88H/51F). Les cancers classant sida ont représenté 84% (117 cas) contre 16 % (22 cas) de cancers non classant sida. Le sarcome de Kaposi a été le plus fréquent (58,99%). Le cancer non classant sida le plus fréquent était le lymphome de Hodgkin (6,47%)(9 cas). Les Lymphomes malins non hodgkiniens et le sarcome de Kaposi étaient corrélés au sexe masculin (p=0,002, p=0,0006). Nous avons enregistré 39 décès (28%) et 34 perdus de vue (24,48%). Conclusion. Les pathologies cancéreuses restent fréquentes au cours du VIH avec une morbi-mortalité importante. Les cancers classant sida demeurent les plus fréquents dans notre contexte.

Objective. To describe the epidemiology of malignancies in people living with HIV at Casablanca. Patients and methods. It was a 13-year retrospective study in the Infectious Diseases Department of Ibn Rochd University Hospital in Casablanca. Included were all HIV-infected patients with histologically documented cancer. Data was collected from Nadis. Data analysis was done on the software epi info. A value of p <0.05 was considered significant. Results. 139 patients had cancerous diseases, an incidence rate of 3.7 patients per 1000 patients' year. The average age was 37 years old. The sex ratio was 1.72 (88H / 51F). Cancer classifying AIDS represented 84% (117 cases) against 16% (22 cases) of non-AIDS-defining cancers. Kaposi's sarcoma was the most common with 58.99%. The most common non-classifying AIDS cancer was Hodgkin's lymphoma with 6.47% (9 cases). Non-Hodgkin's malignant lymphoma and Kaposi's sarcoma were correlated with male sex (p = 0.002, p = 0.0006). We recorded 39 deaths (28%) and 34 lost to follow-up (24.48%). Conclusion. Cancerous pathologies remain frequent during HIV with significant morbidity and mortality. Cancer classifying AIDS remains the most common in our context.

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 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.

Linfoma folicular duodenal primario: reporte de caso y revisión de la literatura / Primary duodenal follicular lymphoma: Case report and literature review

Resumen Los linfomas primarios del tracto gastrointestinal son infrecuentes; sin embargo, son la presentación extranodal más común de los linfomas no Hodgkin. El 30 % de los linfomas no Hodgkin corresponde a linfomas foliculares y, a su vez, cerca del 10 % de los linfomas foliculares se origina en el tracto gastrointestinal. Se han descrito factores de riesgo para el desarrollo de linfomas gastrointestinales como infección por Helicobacter pylori, inmunosupresión posterior a trasplante de órganos sólidos, enfermedad inflamatoria intestinal e infección por virus de la inmunodeficiencia humana (VIH). El linfoma duodenal folicular se reconoció como una variante del linfoma folicular en 2016 según la clasificación de la Organización Mundial de la Salud (OMS), al considerar que se trata de una condición con características biológicas y clínicas particulares. Su diagnóstico suele ser incidental o se pueden presentar síntomas leves e inespecíficos. El grado histológico suele ser bajo y el curso clínico, benigno; por lo que en gran parte de los casos se ha adoptado el manejo expectante como una opción. Otras terapias con similar efectividad son la radioterapia, el uso de rituximab y la inmunoquimioterapia. No existe a la fecha suficiente evidencia para generar un protocolo único de manejo para esta patología.

Abstract Primary gastric lymphomas are rare diseases; however, they are the most common extranodal presentation of non-Hodgkin lymphomas. 30% of non-Hodgkin lymphomas correspond to follicular lymphomas and at the same time, nearly 10% of follicular lymphomas are produced in the gastrointestinal tract. Risk factors for gastric lymphomas such as Helicobacter pylori infection, immunosuppression after solid organ transplantation, inflammatory bowel disease, and human immunodeficiency virus (HIV) infection were described. Follicular duodenal lymphoma was recognized as a variant of follicular lymphoma in 2016 according to the World Health Organization (WHO) classification, considering that it is a condition with special biological and clinical characteristics. Its diagnosis is usually incidental or mild and nonspecific symptoms may occur. The histological grade is usually low, and the clinical course is benign; Therefore, in most cases, expectant treatment has been adopted as an option. Other therapies with similar effectiveness are radiotherapy, the use of rituximab, and immunochemotherapy. There is not enough evidence to date to generate a single management protocol for this pathology.

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.

Esplenectomía laparoscópica en esplenomegalia masiva por linfoma no Hodgkin de zona marginal / Massive laparoscopic splenectomy for marginal zone non-Hodgkin lymphoma / Esplenectomia laparoscópica em esplenomegalia maciça devido a linfoma não-Hodgkin de zona marginal

Resumen: Introducción: si bien la esplenectomía laparoscópica en esplenomegalias masivas y supramasivas constituye un desafío técnico, su realización es factible y segura en centros con equipos con experiencia en cirugía laparoscópica. Objetivo: presentar el primer caso de esplenectomía laparoscópica en esplenomegalia masiva realizada en Uruguay. Caso clínico: se trata de una paciente de 70 años portadora de una pancitopenia periférica, esplenomegalia masiva y diagnóstico realizado por punción de médula ósea de neoplasia linfoproliferativa tipo B de bajo grado, a quien se le indicó la esplenectomía con fines diagnósticos y terapéuticos. La paciente se operó en decúbito lateral derecho a 15 grados, los trócares se colocaron bajo visión directa adaptados al tamaño del bazo que se extendía desde el diafragma hasta el estrecho superior de la pelvis. Se realizó la esplenectomía en un tiempo de 220 minutos, extrayéndose la pieza íntegra y sin haberla colocado en bolsa a través de un hemi Pfannenstiel, protegiendo la pared con un retractor de heridas quirúrgicas. No presentó complicaciones, fue dada de alta a las 48 horas. El hemograma realizado a las 24 horas demostró un aumento de las cifras de todas las series celulares y el informe anatomopatológico diagnosticó un linfoma no Hodgkin de zona marginal. Discusión: la esplenectomía laparoscópica en esplenomegalias masivas requiere de un mayor tiempo quirúrgico, aunque las pérdidas sanguíneas y la estadía hospitalaria son menores en comparación a los procedimientos convencionales, presentando una morbilidad similar. En la experiencia inicial de los equipos quirúrgicos se reporta un porcentaje de conversiones y reingresos cercanos al 30%.

Abstract: Introduction: despite the fact that laparoscopic splenectomy for massive and supramassive splenomegaly constitutes a technical challenge, it is a feasible and safe procedure in the context of institutions with experienced teams in laparoscopic surgery. Objective: to present the first case of laparoscopic splenectomy for massive splenomegaly in Uruguay. Clinical case: the study presents the case of a 70-year-old patient carrier of peripheral pancytopenia, massive splenomegaly and a diagnosis of type B lymphoproliferative neoplasm based on bone marrow aspiration and biopsy, who underwent diagnostic and therapeutic splenectomy. The patient was operated in supine position with a 15-degree tilt, the trocars were placed under direct view, adapted to the size of the spleen which went from the diaphragm until the superior pelvic outlet. Splenectomy was performed in 220 minutes, the entire piece was removed through a hemi Pfannenstiel incision, without placing it in a bag, the wall being protected with a surgical wound retractor. There were no complications and the patient was discharged from hospital 48 hours. The blood count performed after 24 hours evidenced increase in all cell series and the pathology report confirmed diagnosis of marginal zone non- Hodgkin lymphoma. Discussion: laparoscoppic splenectomy in massive splenomegaly requires of a greater surgical time, although blood loss and hospital star are lower when compared to conventional procedures and evidence similar morbility. The initial experience of surgical teams reports 30% of conversions and readmissions.

Resumo: Introdução: embora a esplenectomia laparoscópica em esplenomegalias massivas e supremassivas seja um desafio técnico, sua realização é viável e segura em centros com equipes com experiência em cirurgia laparoscópica. Objetivo: apresentar o primeiro caso de esplenectomia laparoscópica em esplenomegalia maciça realizada no Uruguai. Caso clínico: paciente de 70 anos com pancitopenia periférica, esplenomegalia maciça e diagnóstico feito por punção de medula óssea de neoplasia linfoproliferativa tipo B de baixo grau, com indicação de esplenectomia para fins diagnósticos e terapêuticos. A paciente foi operada em decúbito lateral direito a 15 graus, os trocartes foram colocados sob visão direta adaptados ao tamanho do baço que se estendia do diafragma ao estreito superior da pelve. A esplenectomia foi realizada em um tempo de 220 minutos, retirando-se toda a peça e sem colocá-la em bolsa por meio de uma hemi Pfannenstiel, protegendo a parede com afastador de ferida operatória. Sem apresentar complicações a paciente teve alta após 48 horas. O hemograma realizado 24 horas depois da cirurgia mostrou um aumento no número de todas as séries de células e o laudo anatomopatológico diagnosticou linfoma não Hodgkin de zona marginal. Discussão: a esplenectomia laparoscópica nas esplenomegalias maciças requer um tempo cirúrgico maior, embora as perdas sanguíneas e a permanência hospitalar sejam menores em comparação aos procedimentos convencionais, apresentando morbidade semelhante. Na experiência inicial das equipes cirúrgicas, é relatado um percentual de conversões e readmissões próximo a 30%.

Linfoma de células NK/T subtipo nasal ­ relato de caso / Nasal subtype NK/T cell lymphoma ­ case report

RESUMO O linfoma de células NK/T subtipo nasal tem baixa incidência e alta agressividade, mostrando-se necessário diagnóstico precoce. Tal se mostra desafiador devido à sua manifestação clínica inicial, semelhante a quadro infeccioso de vias aéreas superiores. Por meio deste estudo observacional descritivo, relatam-se casos deste linfoma acometendo paciente masculino e outro feminino, ambos com diagnóstico tardio e com envolvimento de vias aéreas superiores sem resposta à antibioticoterapia. À exame de imagem, apresentaram lesão expansiva em cavidade nasal e o diagnóstico se deu por meio de estudo histológico com marcadores imuno-histoquímicos de células NK atípicas e presença de vírus Epstein-Barr (EBV). PALAVRAS-CHAVE: Linfoma de células NK/T tipo nasal, Epstein-Barr vírus, linfoma não-hodgkin, linfoma de células T periférico

ABSTRACT Nasal subtype NK/T cell lymphoma has a low incidence and high aggressiveness, and early diagnosis is necessary. This is challenging due to its initial clinical manifestation, similar to an upper airway infection. Through this descriptive observational study, we report cases of this lymphoma affecting a male and a female patient, both with late diagnosis and involvement of the upper airways without response to antibiotic therapy. On imaging scan, they presented an expansive lesion in the nasal cavity and the diagnosis was made through histological study with immunohistochemical markers of atypical NK cells and the presence of Epstein-Barr virus (EBV). KEYWORDS: NK/T-cell lymphoma nasal type, Epstein-Barr virus, Lymphoma non Hodgkin, T-cell lymphoma

Linfoma de Burkitt primario de mama durante el embarazo / Primary Burkitt's Lymphoma of the Breast During Pregnancy

El linfoma de Burkitt es una neoplasia altamente agresiva y es un tipo raro de linfoma no Hodgkin localizado. Aunque los niños son los más frecuentemente afectados, en adultos ocurren principalmente durante el embarazo o el puerperio. La mama rara vez constituye la localización primaria del linfoma no Hodgkin. Se presenta un caso de linfoma de Burkitt primario de mama durante el embarazo. Paciente de 37 años con embarazo de 24 semanas quien presentó aumento de volumen difuso de mama derecha. La mama estaba aumentada de tamaño, dolorosa y homogénea con tumoración elástica y firme. La ecografía demostró inflamación difusa con tumoración heterogénea e hipoecoica con contornos ligeramente irregulares, marcadores tumorales estaban normales las pruebas serológicas fueron negativas. La biopsia de la lesión mostró tejido mamario reemplazado por células linfoideas de tamaño mediano con citoplasma basófilo y múltiples vacuolas. Estudios inmunohistoquímicos fueron positivos para el antígeno leucocitario común, CD10, CD20, CD43, Bcl-6. El análisis cromosómico reveló que más del 90 % de las células neoplásicas exhibieron translocación t llevando al diagnóstico final de linfoma de Burkitt de mama. Luego de evaluar las posibilidades terapéuticas y del consentimiento de la paciente se inició tratamiento citostático sistémico. Los linfomas primarios de mama son extremadamente raros. El linfoma de Burkitt primario de la mama es mucho menos común que los otros linfomas. Los métodos de clasificación, detección y tratamiento de esta afección siguen siendo objeto de debates e investigaciones(AU)

The Burkitt's lymphoma is a highly aggressive neoplasm and is a rare type of localized non-Hodgkin lymphoma. Although children are the most frequently affected, in adults they occur mainly during the pregnancy or the puerperium. The breast rarely constitutes the primary location for non-Hodgkin lymphoma. The study of a case of primary Burkitt lymphoma of the breast during pregnancy is presented. This is a 37 year old patient with a 24 week pregnancy who presented a diffuse increase in the volume of the right breast. The breast was enlarged, painful and homogeneous with a firm, elastic mass. The ultrasonography showed diffuse inflammation with a heterogeneous and hypoechoic tumor with slightly irregular contours. The tumor marker values were normal and the serological tests were negative. The biopsy of the lesion showed breast tissue replaced by medium-sized lymphoid cells with basophilic cytoplasm and multiple vacuoles. Immunohistochemically studies were positive for the common leukocyte antigen, CD10, CD20, CD43, Bcl-6. The chromosomal analysis revealed that more than 90 % of neoplastic cells exhibited t translocation leading to the final diagnosis of Burkitt lymphoma of the breast. After evaluating the therapeutic possibilities and the patient's consent, systemic cytostatic treatment was started. Primary breast lymphomas are extremely rare. The primary Burkitt lymphoma of the breast is much less common than other lymphomas. The methods of classification, detection, and the treatment of this condition continue to be the subject of debate and research(AU)

Linfoma no Hodgkin tipo MALT cerebral / Cerebral type MALT non Hodgkin lymphoma

Introducción: Los linfomas no Hodgkin tienen su origen generalmente en el sistema linfoide, existe una gran diversidad de ellos, que hace difícil su clasificación y diagnóstico anatomopatológico definitivo. También hay localizaciones extranodales, como en el cerebro, lo que la hace aún más interesante. Objetivo: Presentar un caso con linfoma marginal cerebral, de diagnóstico muy infrecuente. Caso clínico: Paciente femenina de 67 años de edad con antecedentes patológicos personales de hipertensión arterial y epilepsia, que comenzó a sentirse rígida, con movimientos involuntarios generalizados en el cuerpo, hasta perder la conciencia. Al recuperarse se encontraba somnolienta y con cefalea de moderada intensidad. Se diagnostica un linfoma no Hodgkin tipo MALT cerebral; se realiza tratamiento con el que se revierten los síntomas y se controla la enfermedad. Conclusiones: Es necesario un diagnóstico histopatológico oportuno y certero, con la utilización de los estudios inmunohistoquímicos. Esto permite aplicar un tratamiento precoz y el control de la enfermedad(AU)

Introduction: Non-Hodgkin's lymphomas generally have their origin in the lymphoid system, there is a great diversity of them, which makes their classification and definitive pathological diagnosis difficult. There are also extranodal locations, such as in the brain, which makes it even more interesting. Objective: To present a patient with marginal cerebral lymphoma, of a very infrequent diagnosis. Clinical case: A 67-year-old female patient with a personal pathological history of arterial hypertension and epilepsy, who began to feel rigid, with involuntary movements that generalized to the whole body, until she lost consciousness, when she recovered she was drowsy and with headache of moderate intensity. Brain MALT non-Hodgkin lymphoma is diagnosed, treatment is performed to reverse symptoms, and the disease is controlled. Conclusions: A timely and accurate histopathological diagnosis is necessary, with the use of immunohistochemical studies. This allows for early treatment and disease control(AU)