Compromiso secundario de glándulas mamarias por linfoma folicular / Secondary involvement of breasts by follicular lymphoma

Resumen Las lesiones metastásicas que comprometen la glándula mamaria son excepcionales, ocupando las neoplasias hematolinfoides el segundo lugar en orden de frecuencia en series de casos reporta dos en la literatura con una prevalencia de 0.04% a 1.6% en relación a todos los tumores malignos de la mama, alcanzando una incidencia anual de 0.07%, los cuales corresponden principalmente a linfomas secundarios. El 80% de estos son linfomas B difusos de células grandes, seguido de linfoma folicular y linfoma de la zona marginal. Presentamos una mujer de 60 años con diagnóstico de linfoma folicular que comenzó con una masa perirrenal derecha y ganglios linfáticos ipsilaterales retroperitoneales e inguinales, quien, durante su tratamiento, presentó avance en el estadio clínico con compromiso secundario inusual de ambas glándulas mamarias por esta neoplasia hematolinfoide. Se evaluó el comportamiento biológico de esta enfermedad para comprender los mecanismos fisiopatológicos, mediante el análisis de factores clínicos, histológicos y pronósticos, permitiendo la estadificación definitiva, la cual fue determinante para la elección de la terapia individualizada acorde a las guías de práctica clínica basada en la evidencia científica, impactando positivamente en la evolución médica de la paciente.

Abstract Metastatic lesions involving the breast are exceptional; hematolymphoid neoplasias rank second as per their frequency in case series reported in the literature with a prevalence of 0.04% to 1.6% when consider ing all malignant breast tumors and reaching an annual incidence of 0.07%, mainly accounted for by secondary lymphomas. Eighty percent of them are diffuse, large B cells lymphomas (DLBCL), followed by follicular lymphoma and marginal zone lymphoma. This case is about a 60 year-old woman with a diagnosis of follicular lymphoma, who presented with a right perirenal mass and ipsilateral retroperitoneal and inguinal lymph nodes, whose clinical status progressed during the treatment with unusual secondary involvement of both breasts by hematolymphoid neoplasia. The biological behavior of the condition was evaluated to understand the pathophysiological mecha nisms; this was done analyzing clinical, histologic and prognostic factors that led to a definitive staging, which was key to select the individualized therapy following the clinical practice guidelines based on scientific evidence, with a positive impact on the patient's medical progress.

Secondary involvement of breasts by follicular lymphoma / Compromiso secundario de glándulas mamarias por linfoma folicular

Abstract Metastatic lesions involving the breast are exceptional; hematolymphoid neoplasias rank second as per their frequency in case series reported in the literature with a prevalence of 0.04% to 1.6% when considering all malignant breast tumors and reaching an annual incidence of 0.07%, mainly accounted for by secondary lymphomas. Eighty percent of them are diffuse, large B cells lymphomas (DLBCL), followed by follicular lymphoma and marginal zone lymphoma. This case is about a 60 year-old woman with a diagnosis of follicular lymphoma, who presented with a right perirenal mass and ipsilateral retroperitoneal and inguinal lymph nodes, whose clinical status progressed during the treatment with unusual secondary involvement of both breasts by hematolymphoid neoplasia. The biological behavior of the condition was evaluated to understand the pathophysiological mechanisms; this was done analyzing clinical, histologic and prognostic factors that led to a definitive staging, which was key to select the individualized therapy following the clinical practice guidelines based on scientific evidence, with a positive impact on the patient's medical progress.

Resumen Las lesiones metastásicas que comprometen la glándula mamaria son excepcionales, ocupando las neoplasias hematolinfoides el segundo lugar en orden de frecuencia en series de casos reportados en la literatura con una prevalencia de 0.04% a 1.6% en relación a todos los tumores malignos de la mama, alcanzando una incidencia anual de 0.07%, los cuales corresponden principalmente a linfomas secundarios. El 80% de estos son linfomas B difusos de células grandes, seguido de linfoma folicular y linfoma de la zona marginal. Presentamos una mujer de 60 años con diagnóstico de linfoma folicular que comenzó con una masa perirrenal derecha y ganglios linfáticos ipsilaterales retroperitoneales e inguinales, quien, durante su tratamiento, presentó avance en el estadio clínico con compromiso secundario inusual de ambas glándulas mamarias por esta neoplasia hematolinfoide. Se evaluó el comportamiento biológico de esta enfermedad para comprender los mecanismos fisiopatológicos, mediante el análisis de factores clínicos, histológicos y pronósticos, permitiendo la estadificación definitiva, la cual fue determinante para la elección de la terapia individualizada acorde a las guías de práctica clínica basada en la evidencia científica, impactando positivamente en la evolución médica de la paciente.

Dermatological manifestations of hematologic neoplasms. Part I: secondary specific skin lesions

Abstract Cutaneous manifestations occur during the course of hematologic malignancies and precede, follow, or are late events in relation to the diagnosis. They result from paraneoplastic phenomena, tumor infiltrations, and immunosuppression resulting from the hematologic neoplasia itself or its treatment. The dermatologist must be aware of these conditions, which can help both in the diagnosis of the underlying disease and in the reduction of patient morbidity. This review (part I) addresses skin lesions associated with direct infiltration by systemic hematologic malignancies.

Increased expression of matrix metalloproteinases 2 and 9 as poor prognosis factor for Hodgkin's lymphoma patients

Abstract Objective: The aim of our study was to evaluate the expression of MMP-2 and MMP-9 as a prognostic factor in patients diagnosed with Hodgkin Lymphoma (HL). Methods: In the present study, 45 paraffin biopsies from patients up to 19 years old diagnosed with HL were used in two referral hospitals in the state of Pernambuco, Brazil. Risk groups were classified into favorable and unfavorable, according to Ann Arbor. The expression of matrix metalloproteinases 2 and 9 and their inhibitors was performed by immunohistochemistry (IHC). Data were analyzed using the GraphPad Prism 5 program. Results: MMP-2 intensity pattern was stronger (> 10% of the total field) in patients with stage III/ IV and B symptoms. MMP-2 showed an association with the risk group (p = 0.0388). That is, the stronger the MMP-2 marking, the greater the unfavorable risk. However, for MMP-9 there was no difference in the stronger intensity pattern in relation to stages I/II and III/IV, only in the presence of B symptoms. MMP-9 showed an association with B Symptoms (p = 0.0411). Therefore, patients with B symptoms have higher MMP-9 expression. Conclusion: Our results suggest that MMP-2 expression is associated with HL progression. While MMP-9 expression is related to the clinical worsening of these patients. However, further studies are needed to evaluate the exact role of these proteins in hematologic malignancies.

Virus de Epstein-Barr: más que una mononucleosis infecciosa / Epstein-Barr virus: more than infectious mononucleosis

El virus de Epstein-Barr (VEB) fue el primer virus asociado a neoplasias en humanos. Infecta el 95 % de la población mundial, y aunque usualmente es asintomático, puede causar mononucleosis infecciosa y se relaciona con más de 200.000 casos de neoplasias al año. De igual forma, se asocia con esclerosis múltiple y otras enfermedades autoinmunes. A pesar de ser catalogado como un virus oncogénico, solo un pequeño porcentaje de los individuos infectados desarrollan neoplasias asociadas a VEB. Su persistencia involucra la capacidad de alternar entre una serie de programas de latencia, y de reactivarse cuando tiene la necesidad de colonizar nuevas células B de memoria, con el fin de sostener una infección de por vida y poder transmitirse a nuevos hospederos. En esta revisión se presentan las generalidades del VEB, además de su asociación con varios tipos de neoplasias, como son el carcinoma nasofaríngeo, el carcinoma gástrico, el linfoma de Hodgkin y el linfoma de Burkitt, y la esclerosis múltiple. Adicionalmente, se describen los mecanismos fisiopatológicos de las diferentes entidades, algunos de ellos no completamente dilucidados

Epstein-Barr virus (EBV) was the first virus associated with human cancer. It infects 95% of the world's population, and although it is usually asymptomatic, it causes infectious mononucleosis. It is related to more than 200,000 cases of cancer per year, and is also associated with multiple sclerosis and other autoimmune diseases. Despite being classified as an oncogenic virus, only a small percentage of infected individuals develop EBV-associated cancer. Its persistence involves the ability to alternate between a series of latency programs, and the ability to reactivate itself when it needs to colonize new memory B cells, in order to sustain a lifelong infection and be able to transmit to new hosts. In this review, the general characteristics of EBV are presented, in addition to its association with various types of cancers, such as nasopharyngeal carcinoma, gastric carcinoma, Hodgkin's lymphoma and Burkitt's lymphoma, and multiple sclerosis. Additionally, the pathophysiological mechanisms of the different entities are described, some of them not completely elucidated yet

Immunophenotypic characterization of acute leukemias in Bahia, Brazil

ABSTRACT Objective To characterize the immunophenotypic profile of acute leukemias in the population of the state of Bahia, Brazil. Methods This is a descriptive, retrospective study. From 2014 to 2018, 796 new cases of acute leukemia were evaluated. The data were obtained from analysis of reports and records of tests performed by flow cytometry immunophenotyping. All individuals of all age groups diagnosed as acute lymphoblastic leukemia or acute myeloid leukemia were included in the study. Demographic variables and expression of leukemia antigens were evaluated. Results Most cases were diagnosed as acute myeloid leukemia and 42.7% as acute lymphoblastic leukemia. Significant differences were found in expression of markers in acute leukemias when age groups were compared, as well as in demographic characteristics. B-cell acute lymphoblastic leukemia was more prevalent than cases of T-cell origin. Assessing the aberrant markers in acute myeloid leukemias, the non-acute promyelocytic leukemia group presented expression of CD7 and CD56 as the most frequent ones. In B-cell acute lymphoblastic leukemia, the most frequent aberrant markers were CD66c, CD13 and CD33. Conclusion Significant differences were found as to several antigens when comparing adults and children, and these findings may contribute to future studies correlating the phenotypic profile to genetic characteristics and therapeutic response, including specific antigen therapies, which may be better targeted.

18F-FDG PET/CT Prognostic Role in Diffuse Large B-cell Lymphoma Following Chemotherapy / 中山大学学报(医学科学版)

ObjectiveTo assess the prognostic value of 18F-FDG PET/CT parameters for predicting therapeutic response in diffuse large B-cell lymphoma （DLBCL）. MethodsWe retrospectively analyzed the clinical data and 18F-FDG PET/CT radiomics features of 81 DLBCL patients enrolled between June 2015 and October 2020. Multivariate logistic regression analysis was used to identify the predictive factors for therapeutic response of DLBCL， based on which a predictive model was developed accordingly. The performance of the model was evaluated by receiver operating characteristic （ROC） curves and calibration plots. ResultsDuring the two years after first chemotherapy， 23 patients （28.3%） developed relapse and 58 patients （71.7%） had progression-free survival （PFS）. The analysis for the predictive capability of the binary logistic regression model incorporating the PET/CT features revealed that the imaging features of 18F-FDG PET/CT after chemotherapy were independent prognostic factors for PFS. Among them， SUVTHR-mean2 was the most important factor for predicting therapeutic response in DLBCL patients after chemotherapy， with a cutoff value of 2.00 （AUC=0.81）. Conclusions18F-FDG PET/CT showed a valuable prognostic performance for PFS in DLBCL patients after chemotherapy， with the imaging feature after chemotherapy SUVTLR-mean2 being the optimal independent predictor. Our predictive model of imaging features might have an important prognostic value in assessing the risk of disease progression， guiding the treatment and follow-up protocol， improving therapeutic efficiency and cutting down the medical cost.

Research progress on islet protection and functional maintenance / 器官移植

Islet transplantation is a promising treatment of diabetes mellitus and its complications. Nevertheless, dysfunction post-transplantation, rejection and shortage of donors are the bottleneck issues in the field of islet transplantation. Optimizing the preservation method of pancreas plays a positive role in obtaining a sufficient quantity of effective islets and maintaining their functions. During the culture stage, anti-rejection and anti-apoptosis treatment of islets, including mesenchymal stem cell (MSC), MSC-derived exosomes, anti-apoptosis drugs and gene modification, may become major approaches for islet protection and functional maintenance in clinical islet transplantation. Use of anti-instant blood-mediated inflammatory reaction (IBMIR) drugs after islet transplantation also plays a critical role in protecting islet function. In this article, the whole process from islet preparation to islet transplantation was illustrated, and relevant strategies of islet protection and functional maintenance were reviewed, aiming to provide reference for improving the quality of donors to compensate for the shortage of absolute quantity of donors and elevating the efficiency of islet transplantation.

Effect of Bushen Huoxuetang on Expression of Bax/Bcl-2 and Cleaved Caspase-3 in Nude Mice with Bone Metastasis of Breast Cancer / 中国实验方剂学杂志

ObjectiveTo study the effect of Bushen Huoxuetang on the apoptosis and the expression of B-cell lymphoma （Bcl-2）-associated X protein （Bax）/ Bcl-2 and cleaved cysteine-containing aspartate proteolytic enzyme-3 （cleaved Caspase-3） in the nude mouse model of bone metastasis of breast cancer， and explore the mechanism of Bushen Huoxuetang in inhibiting bone destruction. MethodThirty BALB/c female nude mice were randomly assigned into blank group （n=6） and model group （n=24）. The suspension of 4T1 breast cancer cells was injected into the tibia of mouse right lower limb to establish model of bone metastasis of breast cancer. The successfully modeled nude mice were randomly assigned into model group， Bushen Huoxuetang group， zoledronic acid group， and combined drug group， with 6 mice in each group. Bushen Huoxuetang was administrated at a dose of 36.67 g·kg-1， once a day， and zoledronic acid was administrated by subcutaneous injection at a dose of 100 μg·kg-1， twice a week. The combined drug group was administrated with the same doses of Bushen Huoxuetang group by gavage and zoledronic acid by subcutaneous injection. The mice in the blank group and the model group were administrated with the same volume of distilled water by gavage for 14 days. On the next day at the end of drug administration， the mice were sacrificed by cervical dislocation. The general situation and weight changes of the mice were examined. The right lower limb was collected， and X-ray scanning and hematoxylin-eosin （HE） staining methods were used for observation of pathological changes in the bone. The terminal deoxynucleotidyl transferase dUTP nick-end labeling （TUNEL） was employed to detect the apoptosis of bone tissue in nude mice， and Western blot to determine the expression of Bax/Bcl-2 and cleaved Caspase-3 in the bone tissue. ResultCompared with the blank group， the modeling reduced the body weight （P<0.01） and increased the right lower limb weight of the nude mice （P<0.01）. Compared with the model group， Bushen Huoxuetang， zoledronic acid， and their combination increased the body weight （P<0.01） and decreased the right lower limb weight （P<0.01）. Compared with the blank group， the other groups showed obvious tumor cell atypia， deep nuclear staining， and clear bone metastasis， and the model group showed obvious osteolytic damage in right lower limb and loss of proximal tibia and knee joint. Compared with the model group， Bushen Huoxuetang， zoledronic acid， and their combination reduced the osteolytic lesions in the right lower limb and recovered part of the bone structure， demonstrating an inhibitory effect on bone destruction. The TUNEL assay showed that the model group had lower apoptosis rate of bone metastatic tumor cells than the blank group， Bushen Huoxuetang group， zoledronic acid group， and combined drug group （P<0.01）. Compared with the blank group， the modeling down-regulated the expression of Bax and cleaved Caspase-3 （P<0.01） and up-regulated the expression of Bcl-2 （P<0.01）. Compared with the model group， Bushen Huoxuetang， zoledronic acid， and their combination up-regulated the expression of Bax （P<0.01） and cleaved Caspase-3 （P<0.05， P<0.01） and down-regulated the expression of Bcl-2 （P<0.05， P<0.01）. ConclusionBushen Huoxuetang may inhibit bone destruction in the nude mouse model of bone metastasis of breast cancer by up-regulating the expression of Bax， down-regulating the expression of Bcl-2， activating cleaved Caspase-3， and further inducing apoptosis.

Analysis of influential factors for blood concentrations and adverse drug reaction in the treatment of lymphoma with high-dose methotrexate / 中国药房

OBJECTIVE To discuss the factors affecting the blood concentration of high-dose methotrexate （HD-MTX） and the occurrence of adverse drug reactions （ADR） when treating lymphoma with HD-MTX. METHODS From July 2020 to November 2021， the information of HD-MTX patients who had been monitored for HD-MTX blood drug concentration in the First Affiliated Hospital of Guangdong Pharmaceutical University was collected by retrospective analysis， such as medical record number， age， sex， height， body mass， chemotherapy plan， dosage； test indexes such as alanine transaminase， aspartate transaminase， total bilirubin， creatinine clearance （CrCl）， albumin （ALB） and other indexes were also collected before and after administration. The blood concentrations （c6 h， c24 h， c48 h） of HD-MTX were recorded， drug information of proton pump inhibitors （PPIs） was extracted and used， and ADR occurring within 48 h after administration were all evaluated. Single factor analysis， multiple linear regression and χ2 test were used to analyze the influential factors. RESULTS A total of 133 patients were included in this paper. The results of the single factor analysis of HD-MTX blood drug concentration showed that age， CrCl had an effect on c 6 h （P＜0.05）； age， CrCl and ALB had an effect on c24 h （P＜0.05）； age， body mass index （BMI）， CrCl， combined use of PPIs and ALB had an effect on c48 h （P＜0.05）. The results of multiple linear regression analysis showed that age and CrCl had no effect on c 6 h （P＞0.05）， age was the main influential factor of c 24 h （P＜0.05）， and CrCl and combined use of PPIs were the main influential factors of c48 h （P＜0.05）； the coefficient of variance expansion was between 1 and 3.5， indicating that the analysis results were acceptable. The overall incidence of adverse reactions was 51.13%， of which the blood and lymphatic system reactions were the most common. The results of the influential factors of ADR showed that age， BMI， liver function and CrCl had effect on the incidence of ADR （P＜0.05）. CONCLUSIONS During the process of HD-MTX in the treatment of lymphoma， the patient’s age， CrCl and combined use of PPIs should be considered， and the patient’s blood concentration should be monitored； at the same time， the age of patients， BMI， liver function and CrCl have an impact on the incidence of ADR.

Hallazgos incidentales en cirugía abdominal: masas y sarcomas retroperitoneales / Incidental findings in abdominal surgery: Retroperitoneal masses and sarcomas

Introducción. El retroperitoneo es una estructura que se extiende desde el diafragma hasta la pelvis, está delimitado adelante por el peritoneo parietal, atrás y a los lados por la fascia transversalis y se divide en 9 compartimientos. Se pueden encontrar lesiones primarias o secundarias, cuya evolución clínica varía desde un curso indolente hasta rápidamente progresivo, tanto local como a distancia. Su enfoque, desde el hallazgo hasta el tratamiento, es fundamental para el desenlace oncológico. Objetivo. Analizar la evaluación, el diagnóstico y el tratamiento de las masas retroperitoneales halladas incidentalmente y brindar un algoritmo de manejo. Métodos. Se hizo búsqueda en bases de datos como PubMed y MedicalKey de literatura referentes a tumores retroperitoneales, su diagnóstico y enfoque terapéutico, con el fin de presentar una revisión sobre el abordaje de las masas retroperitoneales y dar nuestras opiniones. Resultados. Se revisaron 43 referencias bibliográficas internacionales y nacionales, y se seleccionaron 20 de ellas, de donde se obtuvieron datos actualizados, recomendaciones de guías internacionales y experiencias nacionales, con lo cual se estructuró este manuscrito. Conclusiones. Las masas retroperitoneales abarcan un espectro de patologías que establecen un reto diagnóstico por su origen embriológico, localización y baja frecuencia. El diagnostico histológico es de vital importancia desde el inicio, para conocer la evolución natural de la enfermedad, y el manejo multidisciplinario en centros de referencia es fundamental para impactar en los desenlaces oncológicos. Existen variadas modalidades terapéuticas, como quimioterapia, radioterapia y resección quirúrgica con estándares oncológicos

Introduction. The retroperitoneum is an structure that extends from the diaphragm to the pelvis, bounded anteriorly by the parietal peritoneum, posteriorly and laterally by the transversalis fascia, and it is divided into 9 compartments. We can find primary or secondary lesions whose clinical evolution varies from an indolent course to a rapidly progressive one, both local and distant. Its approach from discovery to diagnosis and treatment is essential for the oncological outcomes. Objective. To analyze the evaluation, diagnosis and treatment of incidental retroperitoneal masses according to their origin and to provide a management algorithm. Methods. An updated literature search was carried out in databases such as PubMed and Medical Key on retroperitoneal tumors, therapeutic approach and diagnosis, obtaining national and international information to carry out a review article on the approach to retroperitoneal masses.Results. Forty-three international and national bibliographic references were reviewed, based on 20 updated data, recommendations from international guidelines and national experiences were obtained, with which a review and opinion manuscript was structured.Conclusions. Retroperitoneal masses cover a spectrum of pathologies that establish a diagnostic challenge due to their embryological origin, location and low frequency. Histological diagnosis is of vital importance from the beginning to know the natural evolution of the disease and multidisciplinary management in reference centers is essential to impact oncological outcomes. There are many therapeutic modalities from chemotherapy, radiotherapy and surgical resection with oncological standards

Explante de prótese mamária de silicone: reconstrução da mama pela técnica dos retalhos cruzados / Silicone breast prosthesis explant: breast reconstruction using the crossed flap technique

Introdução: Em 1963 Cronin e Gerow introduziram o uso do implante de silicone e seu uso aumentou exponencialmente. Contudo, complicações relacionadas aos implantes surgiram ao longo do tempo. O conjunto de situações adversas ao uso dos implantes de silicone, alimentado pelo crescimento das mídias sociais, culminou em um aumento da retirada definitiva do implante. Muitos casos de explante têm o pedículo inferior comprometido pela lesão dos vasos perfurantes e a técnica dos retalhos cruzados é uma alternativa para a reconstrução das mamas explantadas. Métodos: Foram realizados explantes de silicone com reconstrução imediata da mama sem o uso de um novo implante, motivados por indicação médica ou por desejo próprio do paciente. A técnica dos retalhos cruzados foi utilizada em todos os casos. Ela se vale do cruzamento de retalhos parenquimatosos de pedículo superior, um medial e outro lateral, conforme descrito por Sperli. Resultados: Foram operados 10 casos de 2004 a 2021. O tempo de uso das próteses variou de 3 a 19 anos e a principal motivação para o explante foi contratura capsular. Nenhum caso de necrose foi observado. Conclusões: A técnica dos retalhos cruzados é uma alternativa útil e segura para as cirurgias de reconstrução da mama após explante definitivo.

Introduction: In 1963 Cronin and Gerow introduced the use of the silicone implant and its use increased exponentially. However, complications related to implants emerged over time. The set of adverse situations to the use of silicone implants fueled by the growth of social media culminated in an increase in the permanent removal of the implant. Many cases of explants have the inferior pedicle compromised by injury to the perforating vessels, and the crossed flap technique is an alternative for the reconstruction of explanted breasts. Methods: Silicone explants were performed with immediate breast reconstruction without the use of a new implant, motivated by medical indication or the patients own desire. The crossed flap technique was used in all cases. It uses the crossing of parenchymal patches of the superior pedicle, one medial and one lateral, as described by Sperli. Results: 10 cases were operated from 2004 to 2021. The time of use of the prostheses ranged from 3 to 19 years and the main motivation for the explant was capsular contracture. No cases of necrosis were observed. Conclusions: The crossed flap technique is a useful and safe alternative for breast reconstruction surgeries after definitive explantation.

Inhibidores de bruton tirosina quinasa. ¿Qué necesita saber el oncohematólogo y el cardiólogo? / Bruton tyrosine kinase inhibitors. What does the oncohematologist and the cardiologist need to know?

Resumen Los avances en el conocimiento incorporados en la última década han modificado en gran parte el paradigma del tratamiento de las enfermedades hematológicas malignas. Particularmente la intro ducción de los inhibidores de la Bruton tirosina quinasa (iBTK) y otras drogas blanco junto a nuevos anticuerpos monoclonales se han transformado en los agentes de elección, tanto para la leucemia linfática crónica (LLC) como para otros linfomas "B" periféricos como el linfoma de células del manto (LCM). Los resultados de eficacia frente a la terapia genotóxica son tan exitosos que el fin de la quimio inmunoterapia, sobre todo para la LLC, es ya un postulado reconocido por los principales grupos de investigación. Por otra parte, los nuevos fármacos modificaron el perfil de eventos adversos lo que obligó al desarrollo de nuevas subespecialidades como la cardio-oncología, la cual constituye actualmente un baluarte para el manejo racional de estos pacientes. La presente revisión tiene como objetivo destacar el estado actual del conocimiento sobre estas enfermedades, los principios farmacológicos junto a los nuevos eventos adversos de los iBTK y el invalorable aporte de la cardiología para un correcto tratamiento y control de estos pacientes.

Abstract Advances in knowledge incorporated in the last decade have modified the treatment paradigm in most of the malignant hematological diseases. In particular, the introduction of Bruton tyrosine kinase inhibitors (BTKi) and other target drugs together with new monoclonal antibodies have become agents of choice for both chronic lym phocytic leukemia (CLL) and other peripheral "B" lymphomas such as mantle cell lymphoma (MCL). The results of efficacy against genotoxic therapy are so successful that the end of chemoimmunotherapy, especially for CLL, is already a postulate recognized by the main research groups. On the other hand, the new drugs modified the profile of adverse events, which forced the development of new subspecialties such as cardio-oncology, which currently constitutes a bastion for the rational management of these patients. This review aims to highlight the current state of knowledge on these pathologies, pharmacological principles together with new adverse events of iBTK and the invaluable contribution of cardiology for correct management of these patients.

Linfoma cardíaco primario en el paciente anciano / Primary cardiac lymphoma in the elderly patient

Resumen Se presenta un caso clínico de linfoma cardíaco primario en un paciente anciano, y se explica tanto la aproximación diagnóstica, como el tratamiento que llevó a su remisión completa.

Abstract A clinical case of primary cardiac lymphoma in an elderly patient is presented, and both the diagnostic approach and the treatment that led to its complete remission are explained.

Vasculitis cutánea paraneoplásica asociada a linfomadifuso de célulasB grandes / Paraneoplastic cutaneous vasculitis associated with diffuse large B-cell non-Hodgkin's lymphoma

Resumen Los fenómenos cutáneosparaneoplásicos son heterogéneos, infrecuentes y adquiridos, caracterizados por la presencia subyacente de una neoplasia. La manifestacióncutánea más frecuentemente descripta de la vasculitis paraneoplásica es la púrpura palpable. También puede manifestarse como urticaria, eritema y úlceras de miembros inferiores. Se presenta el caso de un paciente de 38 años con diagnóstico de linfoma difuso de células B grandes y úlceras atípicas de aparición concomitantepor vasculitis leucocitoclástica como fenómeno paraneoplásico. Estas lesiones suelentener un curso paralelo a la neoplasia por lo que al realizar tratamiento de la misma pueden remitir, no así cuando se realiza únicamente tratamiento para las lesiones cutáneas. Remarcamos la importancia de conservar un lecho vital y en estado de granulaciónque acompañeal tratamiento general, para así favorecer una rápida epitelización y prevención de infecciones intercurrentes.

Abstract Paraneoplastic cutaneous phenomena are heterogeneous, infrequent and acquired, characterized by the underlying presence of a neoplasm. The most frequently described cutaneous manifestation of paraneoplastic vasculitis is palpable purpura. It can also manifest as urticaria, erythema and ulcers of the lower limbs. We present the case of a 38-year-old patient with a diagnosis of diffuse large B-cell lymphoma and atypical ulcers of intercurrent onset due to leukocytoclastic vasculitis as a paraneoplastic phenomenon. These lesions usually have a parallel course to the neoplasm, so when the neoplasm is treated they may remit, but not when only the cutaneous lesions are treated. We emphasize the importance of preserving a vital tissue in a state of granulation that accompanies the general treatment, in order to favor a rapid epithelialization and prevention of intercurrent infections.

Linfoma de Hodgkin pulmonar primario

Se presenta el caso de una mujer de 25 años con tos, fiebre, disnea y dolor pleurítico de tres meses de evolución, con una lesión cavitante en la tomografía de tórax. Ella recibió tratamiento antituberculoso en otra institución de salud sin respuesta favorable. Se le realizó biopsia por toracoscopía que reveló presencia de células de Reed Sternberg consistentes con el subtipo de esclerosis nodular del Linfoma de Hodgkin. La paciente cumplió los criterios de linfoma de Hodgkin pulmonar primario, entidad extremadamente infrecuente y muy poco reportada.

We present the case of a 25-year-old woman with a three-month history of cough, fever, dyspnea, pleuritic pain, and a cavitating lesion on chest tomography. She received antituberculous treatment in another health institution without a favorable response. A thoracoscopic biopsy revealed the presence of Reed Sternberg cells consistent with the nodular sclerosis subtype of Hodgkin's lymphoma. The patient met the criteria for primary pulmonary Hodgkin's lymphoma, an extremely infrequent and rarely reported entity. A brief review of the literature is carried out.

Linfoma angioinmunoblástico de células T como imitador de lupus eritematoso sistémico

El linfoma angioinmunoblástico de células T (LAIT) es un linfoma no Hodgkin poco frecuente, puede imitar a enfermedades autoinmunes y es de pobre pronóstico. Se presenta el caso de una mujer de 36 años con 3 años de enfermedad caracterizada por fiebre, artralgias y baja de peso. La paciente fue diagnosticada inicialmente como lupus eritematoso sistémico, pero al no encontrar mejoría con el tratamiento su diagnóstico fue replanteado. En una nueva hospitalización se le identificaron múltiples adenomegalias. Se realizó la biopsia de una de las adenomegalias, la patología fue compatible con LAIT. Se indicó 3 sesiones de quimioterapia, sin embargo, desarrolló falla multiorgánica con desenlace fatal. El LAIT es un reto diagnóstico debido a que puede imitar varias patologías autoinmunes. Es muy importante su sospecha y descarte para iniciar un tratamiento precoz que mejore la sobrevida de los pacientes.

Angioimmunoblastic T-cell lymphoma (LAIT) is a rare non-Hodgkin lymphoma, can mimic autoimmune diseases, and has a poor prognosis. We present the case of a 36-year-old woman with a 3-year illness characterized by fever, arthralgia and weight loss. She was initially diagnosed as systemic lupus erythematosus, but finding no improvement with treatment, her diagnosis was reconsidered. In a new hospitalization, multiple lymph nodes were identified. They performed a biopsy of one of the adenopathies, the pathology was compatible with LAIT. Three chemotherapy sessions were indicated, however, she developed multiple organ failure with a fatal outcome. LAIT is a diagnostic challenge because it can mimic several autoimmune pathologies. Its suspicion and ruling out is very important to initiate early treatment that improves patient survival.

Overall survival in Chilean patients with lymphoma and human immunodeficiency virus: A retrospective cohort study

ABSTRACT Introduction: People living with human immunodeficiency virus (PLWH) still face high morbidity and mortality resulting from lymphoma. Aim: To describe a population of PLWH and lymphoma in a Chilean public hospital and compare the overall survival (OS) with a previously reported cohort from the same institution. Methods: Retrospective single-center cohort study. All the patients diagnosed between 2010 and 2017 were included. Demographic and clinical variables were obtained from medical records. The overall survival (OS) was estimated in treated patients from diagnosis until death or October 2020. The OS was then compared with a cohort of patients diagnosed between 1992 and 2008. Main Results: Eighty-four patients were included. The most common histological types were Burkitt's lymphoma (BL), diffuse large B-cell lymphoma (DLBCL), Hodgkin's lymphoma (HL) and plasmablastic lymphoma (PBL) at 31%, 27%, 21% and 14%, respectively. The three-year OS for the whole cohort of BL, DLBCL, HL and PBL was 58.9%, 65.2%, 47.4%, 76.4% and 50%, respectively. Compared to the cohort of 1992 to 2008, a global increase in the OS was found after excluding HL and adjusting for age and clinical stage (HR 0.38, p = 0.002). However, when the main types were analyzed individually, the increase in the OS was statistically significant only in DLBCL (HR 0.29, p = 0.007). Most patients with DLBCL received CHOP chemotherapy, as in the previous cohort. Conclusion: The OS has improved in this population, despite no major changes in chemotherapy regimens, mainly due to the universal access to antiretroviral therapy.

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.

Linfoma cutâneo primário difuso de grandes células B, tipo perna ­ rápida evolução e desfecho desfavorável: relato de caso / Diffused primary cutaneous lymphoma of large B cells, leg type - quick evolution and unfavorable outcome: case report

Linfomas cutâneos primários são a segunda forma mais comum de linfomas extranodais, sendo os linfomas de células B, (CBCLs) representantes de 20 a 30% dos casos. O linfoma cutâneo difuso de grandes células B, Tipo Perna (PCDLBCL-LT), representa o tipo mais agressivo de CBCLs. Na maioria dos casos, a apresentação clínica é caracterizada por placas ou tumores solitários, ora ulcerados, em uma ou ambas as pernas, de rápido crescimento. O diagnóstico é confirmado através do estudo histopatológico e imunohistoquímico. O tratamento é realizado por meio de quimioterapia e seu prognóstico é reservado com uma sobrevida de 50% a 60% em 05 anos. O objetivo deste trabalho é relatar um caso atendido de linfoma cutâneo primário difuso de grandes células B, tipo perna em um paciente de 75 anos, do sexo masculino com apresentação clínica clássica e desfecho desfavorável, realizar uma revisão bibliográfica do período de 2010 a 2020 na base de dados PUBMED sobre o assunto, dada sua raridade e agressividade ímpar. As informações foram obtidas através de revisão do prontuário, registro fotográfico e revisão da literatura. Por tudo isso, pode-se concluir a importância de estudos multidisciplinares, envolvendo dermatologistas, hematologistas, oncologistas e patologistas para que o diagnóstico e tratamento sejam instituídos o mais precoce possível, visto a raridade e agressividade do PCDLBCL-LT. [au]

Primary cutaneous lymphomas are the second most common form of extranodal lymphomas; with B cell lymphomas (CBCLs) representing 20 to 30% of cases. Diffuse cutaneous large B cell lymphoma, leg type (PCDLBCL-LT), represents the most aggressive type of CBCLs. In most cases, the clinical presentation is characterized by solitary plaques or tumors, sometimes ulcerated, on one or both legs, of rapid growth. The diagnosis is confirmed through histopathological and immunohistochemistry studies. Treatment is carried out through chemotherapy and its prognosis is reserved with a 50% to 60% survival in 5 years. The objective of this work is to report a case of diffuse primary B-cell cutaneous lymphoma, leg type in a 75-year-old male patient with a classic clinical presentation and unfavorable outcome, to perform a literary review from 2010 to 2020 in the PUBMED database on the subject, given its rarity and unique aggressiveness. The data was obtained by reviewing the medical record, photographic record and literature review. For all this, it is possible to conclude the importance of multidisciplinary studies, involving dermatologists, hematologists, oncologists and pathologists so that the diagnosis and treatment are instituted as early as possible, given the rarity and aggressiveness of the PCDLBCL-LT. [au]