Doença falciforme: cuidado com pessoas com úlcera da perna nos serviços de atenção à saúde / Sickle cell disease: caring for people with leg ulcers in heal-thcare services / Enfermedad falciforme: cuidado de personas con úlcera de pierna en los servicios de atención a la salud

Objetivo: identificar o local e os cuidados diretos recebidos por pessoas com úlceras da perna por doença falciforme nos serviços de atenção à saúde. Método: estudo transversal, realizado em 11 centros, no período de agosto de 2019 a abril de 2020. Fizeram parte do estudo 72 pessoas com úlcera da perna ativa. O estudo foi aprovado pelo Comitê de Ética em Pesquisa. Resultado: apresentavam anemia falciforme 91,7% dos participantes, com mediana de três anos de existência da úlcera; 77,8% eram redicivantes; 40,3% compravam os insumos; 66,7% trocavam o próprio curativo no domicílio; 52,8% realizavam uma ou mais trocas diárias; 45,8% dos tratamentos foram prescritos pelo médico; 37,5% eram pomada (colagenase ou antibiótico); 89% não utilizavam compressão para o manejo do edema. Conclusão: a maioria dos participantes não estava inserida na Rede de Atenção à Saúde para o tratamento da úlcera, e não recebia assistência sistematizada e nem insumos apropriados.

Objective: to identify the location and direct care received by people with leg ulcers due to sickle cell disease in health care services. Method: a cross-sectional study carried out in 11 centers from August 2019 to April 2020. The study included 72 people with active leg ulcers. The study was approved by the Research Ethics Committee. Results: a total of 91.7% of the participants had sickle cell anemia, with a median of three years of ulcer existence; 77.8% were recurrent; 40.3% bought the supplies; 66.7% changed their own dressings at home; 52.8% did one or more changes a day; 45.8% of the treatments were prescribed by physician; 37.5% were ointments (collagenase or antibiotics); and 89% did not use compression to manage edema. Conclusion: most of the participants were not included in the Health Care Network for ulcer treatment and did not receive systematized care or appropriate supplies.

Objetivo: identificar el lugar y los cuidados directos recibidos por personas con úlceras de pierna por enfermedad falciforme en los servicios de atención a la salud. Método: estudio transversal, realizado en 11 centros, en el período de agosto de 2019 a abril de 2020. Participaron 72 personas con úlcera de pierna activa. El estudio fue aprobado por el Comité de Ética en Investigación. Resultado: presentaban anemia falciforme 91,7% de los participantes, con una mediana de tres años de existencia de la úlcera; 77,8% eran recidivantes; 40,3% compraban los insumos; 66,7% cambiaban su propio vendaje en el domicilio; 52,8% realizaban uno o más cambios diarios; 45,8% de los tratamientos fueron prescritos por el médico; 37,5% eran pomada (colagenasa o antibiótico); y 89% no utilizaban compresión para el manejo del edema. Conclusión: la mayoría de los participantes no estaba integrada en la Red de Atención a la Salud para el tratamiento de la úlcera, y no recibía asistencia sistematizada ni insumos apropiados.

Histiocitosis de células de Langerhans en un paciente con lesión perianal. Caso clínico / Langerhans cell histiocytosis in a patient with perianal lesion. A case report

La histiocitosis de células de Langerhans es una expresión de células dendríticas mieloides, asociada a un componente inflamatorio significativo y compromiso sistémico variado. La edad más frecuente de presentación es entre 1 y 4 años, y predomina en el sexo masculino. Se comunica el caso de un niño de 5 años de edad cuya forma de presentación fue una lesión granulomatosa con fístula perianal, afectación pulmonar y de oído externo. El abordaje interdisciplinario permitió llegar al diagnóstico, realizar las intervenciones necesarias e iniciar el tratamiento adecuado.

Langerhans cell histiocytosis is an expression of myeloid dendritic cells, associated with a significant inflammatory component and varied systemic involvement. The most common age at presentation is between 1 and 4 years, and it prevails among male subjects. Here we describe the case of a 5-year-old boy who presented with a granulomatous lesion with perianal fistula and lung and external ear involvement. An interdisciplinary approach helped to make a diagnosis, provide the necessary interventions, and start an adequate treatment.

Evaluation of Cytotoxic effect of Moringa peregrina seeds on Oral Cancer, CAL 27, Cell Line and Red Blood Cells Hemolysis / Evaluación del efecto citotóxico de las semillas de Moringa peregrina en el cáncer oral, CAL 27, líneas celulares y hemólisis de glóbulos rojos

Background: Moringa peregrina Forssk is a well-known plant in ethnomedicine due to its widespread uses in various diseases like cough, wound healing, rhinitis, fever, and detoxification. The plant seeds contain compounds that are cytotoxic to many cancer cells. During the therapeutic use of plants via the oral route, some compounds present in the plants may be cytotoxic to normal cell lines and red blood cells. Objective: This study was the first report of investigation of the cytotoxic profile on oral cancer, CAL 27, cell line, and hemolytic activities on human erythrocytes of Moringa peregrina seeds ethanolic extract (MPSE). Methods: MPSE was screened for its cytotoxic effect against oral cancer, CAL 27, cell line using 3-(4, 5-dimethylthiazol-2-yl)-2, 5,-diphenyltetrazolium bromide (MTT) assay. The toxicity of MPSE on human erythrocytes was determined by in vitro hemolytic assay. Results: MPSE showed significant anti-proliferative activity against oral cancer, CAL 27 cell line at lower concentrations with half maximal inhibitory concentration (IC50) value of 21.03 µg/mL. At 1,000 µg/ml of MPSE, the maximum hemolysis was found to be 14.3% which is within safer limit. Conclusions: This study revealed a potential anti-oral cancer of MPSE and provided a baseline for its potential use in oral cancer treatment with minimum hemolytic effect on human RBCs.

La Moringa peregrina Forssk es una planta muy conocida en etnomedicina debido a sus usos generalizados en diversas enfermedades como la tos, la cicatrización de heridas, la rinitis, la fiebre y la desintoxicación. Las semillas de la planta contienen compuestos citotóxicos para muchas células cancerosas. Durante el uso terapéutico de las plantas por vía oral, algunos compuestos presentes en ellas pueden ser citotóxicos para las líneas celulares normales y los glóbulos rojos. Objetivo: Este estudio fue el primer informe de investigación del perfil citotóxico sobre el cáncer oral, CAL 27, línea celular, y las actividades hemolíticas en eritrocitos humanos del extracto etanólico de semillas de Moringa peregrina (MPSE). Métodos: Se examinó el efecto citotóxico del MPSE contra la línea celular de cáncer oral CAL 27 mediante el ensayo con bromuro de 3-(4, 5-dimetiltiazol-2-il)-2, 5,-difeniltetrazolio (MTT). La toxicidad del MPSE sobre los eritrocitos humanos se determinó mediante un ensayo hemolítico in vitro. Resultados: MPSE mostró una actividad antiproliferativa significativa contra el cáncer oral, línea celular CAL 27 a concentraciones más bajas con un valor de concentración inhibitoria media máxima (IC50) de 21,03 µg/mL. A 1.000 µg/ml de MPSE, la hemólisis máxima fue del 14,3%, lo que está dentro del límite de seguridad. Conclusiones: Este estudio reveló un potencial anticancerígeno oral de MPSE y proporcionó una base para su uso potencial en el tratamiento del cáncer oral con un efecto hemolítico mínimo en los glóbulos rojos humanos.

Network meta-analysis of pembrolizumab as monotherapy and in combination with chemotherapy for first-line treatment of recurrent or metastatic head and neck squamous cell carcinoma / Metanálise em rede comparativa de pembrolizumabe como monoterapia e em combinação com quimioterapia para tratamento de primeira linha de carcinoma de células escamosas da cabeça e pescoço recorrente ou metastático

Pembrolizumab monotherapy or in combination with chemotherapy is approved as first-line treatment in recurrent/metastatic head and neck squamous cell carcinoma (R/M HNSCC) based on improved overall survival (OS) versus EXTREME regimen in the KEYNOTE-048 trial. The clinical outcomes of pembrolizumab were compared with other recommended first-line treatments in R/M HNSCC in this study through a Bayesian network meta-analysis. A systematic literature review was conducted in July 2022, from which six trials that matched the KEYNOTE-048 patient eligibility criteria were included in the network. The OS and progression-free survival (PFS) outcomes were compared in the approved pembrolizumab indication (i.e., total population for pembrolizumab in combination with chemotherapy and combined positive score [CPS] ≥ 1 population for pembrolizumab monotherapy). A significant OS improvement was observed for pembrolizumab in combination with chemotherapy and pembrolizumab monotherapy versus EXTREME regimen (hazard ratio, 95% credible interval: 0.72, 0.60-0.86; 0.73, 0.60-0.88), platinum+5- FU (0.58, 0.43-0.76; 0.58, 0.44-0.78), and platinum+paclitaxel (0.53, 0.35-0.79; 0.53, 0.35-0.81), respectively. A non-significant numeric trend in OS improvement was observed versus the TPEx regimen. PFS was comparable with most first-line treatments and was improved versus platinum+5-FU (0.48, 0.36-0.64; 0.59, 0.45-0.79). Additional analyses in higher CPS subgroups also showed consistent results. Overall, our study results showed an improvement in OS outcomes versus alternative first-line treatments, consistent with the findings of the KEYNOTE-048 trial. These data support using pembrolizumab as a suitable firstline treatment option in R/M HNSCC.

Pembrolizumabe em monoterapia ou em combinação com quimioterapia é aprovado como tratamento de primeira linha em carcinoma de células escamosas recorrente/metastático de cabeça e pescoço (CECCP R/M) com base na melhora da sobrevida global (OS), em comparação com o esquema EXTREME no estudo KEYNOTE-048. Esse estudo comparou os resultados clínicos de pembrolizumabe com outros tratamentos recomendados de primeira linha em CECCP R/M por meio de uma metanálise de rede bayesiana. Uma revisão sistemática da literatura foi conduzida em julho de 2022, a partir da qual seis ensaios clínicos que atendiam aos critérios de elegibilidade de pacientes do KEYNOTE-048 foram incluídos na rede. Os desfechos de OS e sobrevida livre de progressão (PFS) foram comparados na indicação de pembrolizumabe (população total para pembrolizumabe em combinação com quimioterapia e população com escore positivo combinado [CPS] ≥ 1 em monoterapia com pembrolizumabe). Foi observada melhora significativa na OS para pembrolizumabe em combinação com quimioterapia e monoterapia com pembrolizumabe versus o esquema EXTREME (razão de risco, intervalo de confiança de 95%: 0,72, 0,60-0,86; 0,73, 0,60-0,88), platina+5-FU (0,58, 0,43-0,76; 0,58, 0,44-0,78) e platina+paclitaxel (0,53, 0,35-0,79; 0,53, 0,35-0,81), respectivamente. Uma tendência numérica não significativa de melhoria na OS foi observada em relação ao esquema TPEx. A PFS foi comparável com a maioria dos tratamentos de primeira linha e melhor em relação à platina+5-FU (0,48, 0,36-0,64; 0,59, 0,45-0,79). Análises adicionais em subgrupos com CPS mais elevado também mostraram resultados consistentes. No geral, os resultados de nosso estudo mostraram melhora nos desfechos de OS em comparação aos tratamentos de primeira linha alternativos, consistentes com os achados do estudo KEYNOTE-048. Esses dados apoiam o uso de pembrolizumabe como opção de tratamento em primeira linha em pacientes com CECCP R/M.

Mycobacterium tuberculosis y virus linfotrópico humano, una co-infección fatal

La tuberculosis es una infección de alta incidencia en Latinoamérica. Su presentación como infección activa está determinada por factores de riesgo del hospedero. Comunicamos el caso clínico de una mujer joven que presentó una forma grave de tuberculosis pulmonar. Al explorar sus factores de riesgo se confirmó un estado de inmunosupresión profundo, causado por un linfoma de células T, asociada a una co-infección por virus linfotrópico T humano tipo 1. Se destacan los aspectos microbiológicos y de pronóstico de la co-infección de Mycobacterium tuberculosis y HTLV-1

Tuberculosis is a high-incidence infection in Latin America. Its presentation as an active infection is determined by risk factors in the host. We report the case of a young woman who presented a severe form of pulmonary tuberculosis. When exploring her risk factors, a profound state of immunosuppression was found, caused by T-cell lymphoma, associated with co-infection with human lymphotropic virus. Microbiological and prognostic aspects of Mycobacterium tuberculosis and HTLV-1 co-infection are highlighted.

Síndrome visual informático en pacientes menores de 35 años

Introducción: El síndrome visual informático o fatiga visual digital es una enfermedad causada por el cansancio ocular que provoca el pasar mucho tiempo frente a una pantalla. Objetivo: Diagnosticar el síndrome visual informático en pacientes menores de 35 años atendidos en la consulta de refracción. Métodos: Se realizó un estudio descriptivo prospectivo y transversal de pacientes atendidos en la consulta de refracción del Policlínico Especialidades del Hospital Provincial Docente Clínicoquirúrgico Saturnino Lora, durante el periodo de abril a junio de 2022. Resultados: Predominaron los pacientes en las edades comprendidas entre 26 y 35 años y del sexo femenino; los síntomas más frecuentes fueron el cansancio visual, ardor ocular, sensación de ojo seco, visión borrosa de cerca, ojo rojo y el dolor de cabeza después del esfuerzo visual. Los dispositivos digitales más usados fueron el celular y la computadora con un tiempo de uso de una a tres horas, destacándose este último con un tiempo superior a 4 horas. Los defectos refractivos constituyeron la principal causa de limitaciones visuales. Los pacientes con síndrome visual informático y alguna ametropía sin corrección fueron los que mayor cantidad de síntomas tuvieron, seguidos de los pacientes corregidos inadecuadamente. Conclusiones: Este síndrome afecta en gran medida a la población más joven. El adecuado interrogatorio y la incorporación de los procedimientos correctos en el estudio optométrico diario permitió el diagnóstico de tal síndrome en los pacientes atendidos y la corrección óptica pertinente.

Introduction: Digital visual syndrome or digital visual fatigue is a disease caused by the ocular fatigue provoked by spending much time in front of a screen. Objective: To diagnose the digital visual syndrome in patients under 35 years assisted in the refraction service. Methods: A prospective descriptive and cross-sectional study of patients assisted in the refraction service of the Specialties Polyclinic in Saturnino Lora Teaching Clinical Surgical Provincial Hospital, was carried out from April to June, 2022. Results: There was a prevalence of patients aged 26 and 35 and female sex; the most frequent symptoms were visual fatigue, ocular burning, dry eye sensation, closely blurred vision, red eye and headache after visual effort. The most used digital devices were the cellphone and the computer with a time of use from one to three hours, with emphasis in the last one with more than 4 hours. The refractive defects constituted the main cause of visual limitations. The patients with digital visual syndrome and some type of ametropia without correction were those with more symptoms, followed by the patients inadequately corrected. Conclusions: This syndrome affects the youngest population to a great extent. The appropriate interrogation and the incorporation of correct procedures in the daily optometric study allowed the diagnosis of such a syndrome in the assisted patients and the pertinent optic correction.

Carcinoma basocelular de vulva: relato de caso / Basal cell carcinoma of the vulva: case report

Introdução: O carcinoma basocelular (CBC) de vulva é uma condição rara que corresponde a menos de 0,4% dos casos de CBC e de 2% a 4% das neoplasias de vulva. O CBC de vulva é mais comum entre mulheres brancas, multíparas e na pósmenopausa, especialmente na sétima década de vida. O objetivo é relatar um caso de CBC de vulva no qual discutiram-se os aspectos do diagnóstico e tratamento. Relato de Caso: Mulher de 63 anos de idade, G1P1A0, chega ao consultório em janeiro de 2022 para tratamento de lesão persistente em vulva. Realizou-se biópsia incisional que mostrou tratar-se de provável carcinoma basocelular nodular com invasão da derme. A paciente submeteu-se a uma ressecção do tumor com margens macroscópicas livres e sutura primária. A cirurgia não teve complicações no pré-operatório e no pós-operatório. O histopatológico da peça cirúrgica mostrou tratar-se de carcinoma basocelular nodular com área irregular, plana, branco, medindo 0,7x0,4cm, com as margens laterais distando 7,0 e 5,0mm e profundas, 5,9mm; todas livres. Conclusão: O caso relatado é raro, tendo sido o tratamento de ressecção cirúrgica do CBC de vulva com margens bem-sucedido. Catorze meses após a cirurgia, a paciente encontra-se sem evidências de recidiva local ou regional.

Introduction: Basal cell carcinoma (BCC) of the vulva is a rare condition that accounts for less than 0.4% of BCC cases and 2% to 4% of vulvar neoplasms. BCC of the vulva is more common among white, multiparous and postmenopausal women, especially in the seventh decade of life. The aim is to report a case of BCC of the vulva in which aspects of diagnosis and treatment were discussed. Case report: A 63-year-old woman, G1P1A0, arrives at the office in January 2022 for treatment of a persistent lesion on her vulva. An incisional biopsy was performed and showed that it was likely nodular basal cell carcinoma with invasion of the dermis. The patient underwent tumor resection with free macroscopic margins and primary suture. The surgery had no complications preoperatively or postoperatively. The histopathology of the surgical specimen showed that it was a nodular basal cell carcinoma with an irregular, flat, white area, measuring 0.7x0.4cm, with the lateral margins 7.0 and 5.0mm apart and 5.9mm deep; all free. Conclusion: The reported case is rare, with surgical resection of BCC of the vulva with margins being successful. Fourteen months after surgery, the patient has no evidence of local or regional recurrence.

Tumor filoide borderline gigante e recidivante: ressecção e reconstrução da mama / Recurrent giant borderline phyllodes tumor: resection and breast reconstruction

O tumor filoide é uma neoplasia fibroepitelial rara que representa 0,3 a 1% de todas as neoplasias mamárias. De acordo com a classificação histopatológica, 12 a 26% são do tipo borderline e aproximadamente 15% desses tumores recorrem após excisão cirúrgica. O tratamento recomendado para todos os tipos de tumor filoide é a excisão cirúrgica, e no caso de tumores gigantes o tratamento deve ser multidisciplinar. Apresentamos o caso de uma mulher de 46 anos com tumor filoide na mama esquerda que recorreu 4 anos após a excisão cirúrgica. O estudo anatomopatológico qualificou-o como tumor gigante e o estudo histopatológico relatou tumor filoide borderline. Foi submetida a excisão cirúrgica com mastectomia esquerda e reconstrução mamária com retalho de grande dorsal mais enxerto de gordura. A paciente apresentou evolução favorável sem recidiva. Concluindo, o tumor filoide gigante borderline recorrente é raro e seu manejo cirúrgico representa um desafio tanto na excisão quanto na reconstrução mamária.

Phyllodes tumor is a rare fibroepithelial neoplasm that represents 0.3 to 1% of all breast neoplasms. According to histopathologic classification, 12 to 26% are borderline type and approximately 15% of these tumors recur after surgical excision. The recommended treatment for all types of phyllodes tumor is surgical excision, and in the case of giant tumors the treatment should be multidisciplinary. We present the case of a 46-yearold woman with a phyllodes tumor in the left breast that recurred 4 years after surgical excision. The anatomopathological study qualified it as a giant tumor and the histopathological study reported a borderline phyllodes tumor. She underwent surgical excision with left mastectomy and breast reconstruction by means of a latissimus dorsi flap plus fat graft. The patient presented a favorable evolution without recurrence. In conclusion, the recurrent giant borderline phyllodes tumor is rare and its surgical management represents a challenge both in breast excision and reconstruction.

Associação do retalho miocutâneo do músculo peitoral maior e da mamaplastia em T invertido para reconstrução de extenso defeito da parede torácica: relato de caso / Association of pectoralis major myocutaneous flap and inverted T mammoplasty for reconstruction of extensive chest wall defect: case report

Introdução: Em 1977, a partir dos estudos anatômicos de McCraw et al., passou-se a utilizar o músculo peitoral maior como retalho miocutâneo em ilha. O presente artigo descreve um caso de reconstrução de um defeito da parede anterior do hemitórax direito através do retalho miocutâneo peitoral maior em ilha ipsilateral. Relato do Caso: A.E.S., de 66 anos, sexo masculino foi submetido a ressecção ampla de um carcinoma basocelular infiltrativo recidivante de 13,0 x 8,0cm da região paraesternal direita. O retalho miocutâneo foi transposto através de tunelização subcutânea e as cicatrizes posicionadas em forma de mamaplastia em T invertido. Conclusão: A presente tática cirúrgica é de fácil execução para cirurgiões habituados com reconstrução mamária, apresenta tempo cirúrgico curto e resultado estético-funcional satisfatório.

Introduction: In 1977, based on anatomical studies by McCraw et al., the pectoralis major muscle began to be used as an island myocutaneous flap. The present article describes a case of reconstruction of a defect in the anterior wall of the right hemithorax using the pectoralis major myocutaneous flap in an ipsilateral island. Case Report: AES, 66 years old, male, underwent wide resection of a recurrent infiltrative basal cell carcinoma measuring 13.0 x 8.0 cm in the right parasternal region. The myocutaneous flap was transposed through subcutaneous tunneling and the scars were positioned in the shape of an inverted T mammoplasty. Conclusion: This surgical tactic is easy to perform for surgeons accustomed to breast reconstruction, has a short surgical time, and has satisfactory aesthetic-functional results.

Número de células CD34+ circulantes como predictor de recolección exitosa de progenitores hematopoyéticos por aféresis en trasplante autólogo / Number of circulating CD34+ cells as a predictor of successful collection of hematopoietic progenitors by apheresis in autologous transplantation

RESUMEN Introducción. El trasplante autólogo de células progenitoras hematopoyéticas es una terapia eficaz en neoplasias malignas hematológicas. El número de células que CD34+ en sangre periférica es el mejor predictor del rendimiento de recolección de células progenitoras hematopoyéticas. Objetivo. Determinar el número de células CD34+ en sangre periférica asociado al éxito de recolección de progenitores hematopoyéticos por aféresis en trasplante autólogo. Métodos. Se evaluó retrospectivamente los datos de 236 procedimientos de aféresis de células progenitoras hematopoyéticas para el trasplante autólogo en el Hospital Edgardo Rebagliati Martins (Lima, Perú) de julio del 2020 a julio del 2023. Se utilizó la curva ROC (características operativas del receptor) para determinar el número de células CD34+ en sangre periférica necesario para lograr una recolección por aféresis ≥ 2 x 106 células CD34+/kg. Resultados. El 61% fueron hombres, con mediana de edad de 58 años, el valor de corte fue de 18,38 células CD34+/μL (sensibilidad de 94,1% y especificidad de 96,9%). Conclusión. El número de células CD34+ sangre periférica para una recolección exitosa de células progenitoras hematopoyéticas para el trasplante autólogo fue de 18,38 células CD34+/μL.

ABSTRACT Introduction. Autologous hematopoietic progenitor cell transplantation is an effective therapy in hematological malignancies, the number of CD34+ cells in peripheral blood is the best predictor of hematopoietic progenitor cell harvesting performance. Objective. To determine the number of CD34+ cells in peripheral blood associated with the successful collection of hematopoietic progenitors by apheresis in autologous transplantation. Methods. The data of 236 hematopoietic progenitor cell apheresis procedures for autologous transplantation at the Edgardo Rebagliati Martins Hospital (Lima, Peru) were retrospectively evaluated from July 2020 to July 2023. The ROC (receiver operating characteristics) curve was used to determine the number of CD34+ cells in peripheral blood necessary to achieve an collection by apheresis ≥ 2 x 106 CD34+ cells/kg. Results. 61% were men, with a median age of 58 years, the cut-off value was 18.38 CD34+ cells/μL (sensitivity of 94.1% and specificity of 96.9%). Conclusion. The number of peripheral blood CD34+cells for successful collection of hematopoietic progenitor cells for autologous transplantation was 18.38 CD34+ cells/μL.

Production of dendritic cell vaccines using different methods with equivalent results: Implications for emerging centers

Introduction Dendritic cell (DC) vaccines have demonstrated good efficacy in preventing relapse and in increasing survival of patients affected by a variety of both solid and hematological tumors. Most protocols used to generate these cells involve the automated separation of peripheral blood monocytes from patients. This approach requires specialized equipment, which elevates the cost of this type of therapy, potentially limiting the widespread access to patients. Method: In this study, we compare the yield and quality of dendritic cells generated from monocytes and isolated by an automated method or by manual methods using gradient centrifugation. Results The results demonstrate the equivalence of the 3 methods in relation to the yield and final quality of the product, however with considerable differences between the costs of these procedures. In addition, this study also demonstrates the feasibility of the antigenic pulse with autologous tumor cell lysates, constituting a source of antigens, not only easily obtained and manipulated, but also specific to the patient's tumor. Conclusion These findings may have important implications for emerging centers interested in using this medical approach and potentially increase the access of a greater number of patients to this therapeutic option.

Serial values of hematologic variables and deep venous thrombosis: Red cell distribution width is associated with deep venous thrombosis

Abstract Introduction As 30 to 50% of deep venous thrombosis (DVT) cases remain idiopathic, an increased focus on hematologic variables may therefore reveal novel correlates of DVT. Very few studies have investigated the association of hematological parameters with DVT and the causal relationship between them is still to be elucidated. Therefore, we aimed to investigate the association between serial values of hematologic variables and DVT. Methods Complete blood count parameters were serially measured at baseline and then at approximately 3-month intervals for 12 months in 152 adults with the first episode of DVT and 152 age- and sex-matched controls. The odds ratio (OR) with the 95% confidence interval (95%CI) was calculated as a measure of association between hematological parameters and DVT. Results The red cell distribution width (RDW) was the only hematologic variable which showed an independent and consistent association with DVT at all time points (multivariable-adjusted OR [95%CI] 3.38 [1.28 - 8.91] at baseline, 2.24 [0.85 - 5.92] at 3 months and 2.12 [0.81 - 5.55] at 12 months for RDW > 14.0%). This association was higher for provoked DVT than unprovoked DVT and for DVT plus pulmonary embolism than DVT alone. No significant correlation was found between the high RDW and classical thrombotic risk factors, except malignancy. Conclusions We demonstrated an independent and consistent association of the high RDW with the first episode of DVT in adult patients. The study was probably underpowered to evaluate the association between the high RDW and recurrent DVT. Further large studies with long follow-up are needed to confirm this association.

Consensus of the Brazilian Association of Hematology, Hemotherapy and Cellular Therapy (ABHH) and the Brazilian Ministry of Health - General management of blood and blood products on the tests necessary for the release of exceptional medicines for sickle cell disease

Abstract To date, hydroxyurea is the only effective and safe drug that significantly reduces morbidity and mortality of individuals with Sickle cell disease. Twenty years of real-life experience has demonstrated that hydroxyurea reduces pain attacks, vaso-occlusive events, including acute chest syndrome, the number and duration of hospitalizations and the need for transfusion. The therapeutic success of hydroxyurea is directly linked to access to the drug, the dose used and adherence to treatment which, in part, is correlated to the availability of hydroxyurea. This consensus aims to reduce the number of mandatory exams needed to access the drug, prioritizing the requesting physician's report, without affecting patient safety.

Síndrome torácico agudo por drepanocitosis como causa de muerte súbita: Reporte de un caso y revisión de literatura / Acute chest syndrome due to sickle cell disease as a cause of sudden death: Case report and literature review

Resumen Introducción: La anemia de células falciformes se define como la hemoglobinopatía estructural autosómica recesiva más prevalente en todo el mundo, puede presentar fenómenos vasooclusivos y la anemia hemolítica que son los responsables de las principales complicaciones de esta enfermedad, una de estas es el síndrome torácico agudo, siendo una causa importante de morbilidad y mortalidad en estos pacientes. Objetivo: correlacionar los hallazgos de la autopsia médico legal, con las generalidades de la fisiopatología de la anemia de células falciformes actualizadas y disponibles. Materiales y métodos: Se realizó el análisis del protocolo de la autopsia médico legal efectuada, y revisión bibliográfica en un caso de una femenina de 37 años que en apariencia padecía de "dolor por nervio ciático", quien falleció en su casa de habitación, tras presentar un dolor intenso localizado en la espalda y disnea. Tras la autopsia médico legal se estableció como causa de muerte el síndrome torácico agudo por drepanocitosis. Conclusiones: La anemia de células falciformes se debe tener presente al momento de la valoración médico legal como una posible causa de muerte, para ello es necesario conocer la fisiopatología de esta enfermedad, y poder identificar dichas afectaciones al momento de realizar la autopsia médico legal, para esto es necesario contar con todas las herramientas posibles para poder realizar un diagnóstico certero.

Abstract Objective: To correlate the findings of the medical legal autopsy carried out with the updated and available generalities of the pathophysiology of sickle cell anemia. Materials and methods: The analysis of the protocol of the medical-legal autopsy carried out, and a bibliographic review, were carried out. A 37-year-old female who apparently suffered from "sciatic nerve pain," who died in her home after experiencing intense pain located in her back and dyspnea. After the medical-legal autopsy, acute chest syndrome due to sickle cell disease was established as the cause of death. Conclusions: Sickle cell anemia must be kept in mind at the time of the medical-legal evaluation as a possible cause of death, for this it is necessary to know the pathophysiology of this disease, and to be able to identify these effects when performing the Medical- Legal Autopsy, for this It is necessary to have all the possible tools to be able to make an accurate diagnosis.

Resección de metástasis pancreáticas: análisis de resultados quirúrgicos y oncológicos. Estudio de cohorte retrospectiva / Pancreatic resections for metastases in the pancreas: analysis of surgical and oncologic outcomes. A retrospective cohort study

RESUMEN Antecedentes: las metástasis pancreáticas, si bien son poco frecuentes, representan una entidad clínica cuyo diagnóstico probablemente se incrementará en el futuro por el aumento de los programas de seguimiento oncológico. Objetivo: describir los resultados quirúrgicos y oncológicos de una serie de pacientes operados por metástasis pancreáticas. Materiales y métodos: se realizó un estudio de cohorte retrospectivo, descriptivo, multicéntrico, de los pacientes sometidos a resecciones pancreáticas por metástasis entre enero de 2016 y diciembre de 2022, en tres efectores de salud por el mismo grupo quirúrgico. Resultados: fueron operados 19 pacientes, con una media de edad de 59 años (45-79), 11 de sexo femenino, en buen estado general y sin otra evidencia de enfermedad oncológica. El origen de los tumores primarios fue 14 en riñón (7 diagnosticados durante el seguimiento), uno carcinoma mamario, uno melanoma, uno testicular, uno colorrectal y uno de cuello de útero. Las técnicas quirúrgicas empleada fueron: 7 esplenopancreatectomías (5 videolaparoscópicas y 2 convencionales), 4 enucleaciones (3 convencionales y 1 videolaparoscópica), 3 duodenopancreatectomías cefálicas convencionales, 2 duodenopancreatectomías totales convencionales, 2 pancreatectomías centrales convencionales, y una pancreatectomía corporocaudal con preservación del bazo. No se registró mortalidad operatoria (dentro de los 90 días posoperatorios), y presentaron una supervivencia global y libre de enfermedad de 58 y 53 meses, respectivamente. Conclusión: la resección de metástasis pancreáticas, en casos seleccionados, con un abordaje multidisciplinario, y en centros de alto volumen de patología hepatobiliopancreática, es segura y permite buenos resultados oncológicos y de supervivencia global.

ABSTRACT Background: Pancreatic metastases are rare but are likely to be diagnosed more frequently in the future due to the increase in oncology surveillance programs. Objective: The aim of this study was to describe the surgical and oncologic outcomes of a series of patients undergoing surgery for pancreatic metastases. Materials and methods: We conducted a retrospective, descriptive, and multicenter cohort study on patients who underwent pancreatic resections for metastases in the pancreas by the same surgical group between January 2016 and December 2022 in three healthcare providers. Results: A total of 19 patients were operated on, mean age was 59 years (45-79), and 11 were women with good performance status and no other evidence of oncologic disease. Clear cell renal cell carcinoma was the primary tumor in 14 cases (7 diagnosed during surveillance), and the remaining primary tumors were one case of breast ductal carcinoma, one testicular cancer, one colorectal cancer, one melanoma and one cervical cancer. The surgical techniques used were pancreatectomies and splenectomies in 7 patients (5 via laparoscopy and 2 conventional procedures), 4 enucleations (3 conventional procedures and 1 laparoscopic surgery), 3 conventional cephalic pancreaticoduodenectomies, 2 conventional central pancreatectomies and one spleen-preserving distal pancreatectomy. No deaths were reported within 90 days of surgery, and overall survival and disease-free survival were 58 and 53 months, respectively. Conclusion: Resection of pancreatic metastases is safe and provides good oncologic outcomes and overall survival when performed with a multidisciplinary approach in centers with a high volume of hepatobiliary and pancreatic surgeries and in selected cases.

Tumor neuroectodérmico maligno del tracto gastrointestinal: a propósito de un caso / Malignant neuroectodermal tumor of the gastrointestinal tract: case report

El tumor neuroectodérmico maligno del tracto gastrointestinal es una neoplasia rara con pocos casos reportados en la literatura, especialmente en América Latina. Descrito por primera vez en 2003, se trata de una entidad sin tratamiento estandarizado y de pobre pronóstico. Se presenta el caso de una paciente de 22 años de edad que acude a la consulta por dolor abdominal, anemia y masa abdominal palpable. Luego de estudios pertinentes se decide la conducta resectiva y el posterior tratamiento oncológico. (AU)

Malignant gastrointestinal neuroectodermal tumor (GNET), formerly known as clear cell sarcoma of the gastrointestinal tract, is an extremely rare tumor of mesenchymal origin, which presents great microscopic and molecular similarity to clear cell sarcoma found in other parts of the body, such as tendons and aponeurosis. It is characterized by its rapid evolution, high recurrence rate and frequent diagnosis as metastatic disease.1,2 (AU)

Trasplante de médula ósea haploidéntico en un paciente pediátrico con síndrome de Wiskott-Aldrich. A propósito de un caso / Haploidentical bone marrow transplantation in a pediatric patient with Wiskott-Aldrich syndrome. A case report

El síndrome de Wiskott-Aldrich es un error innato de la inmunidad de herencia ligada al cromosoma X, producido por variantes en el gen que codifica la proteína del síndrome de Wiskott-Aldrich (WASp). Reportamos el caso clínico de un paciente de 18 meses con diagnóstico de Wiskott-Aldrich que no presentaba donante antígeno leucocitario humano (HLA) idéntico y recibió un trasplante de células progenitoras hematopoyéticas (TCPH) con donante familiar haploidéntico. La profilaxis para enfermedad de injerto contra huésped incluyó ciclofosfamida (PT-Cy). El quimerismo del día +30 fue 100 % del donante y la evaluación postrasplante de la expresión de la proteína WAS fue normal. Actualmente, a 32 meses del trasplante, presenta reconstitución hematológica e inmunológica y quimerismo completo sin evidencia de enfermedad injerto contra huésped. El TCPH haploidéntico con PT-Cy se mostró factible y seguro en este caso de síndrome de WiskottAldrich en el que no se disponía de un donante HLA idéntico.

Wiskott-Aldrich syndrome (WAS) is an X-linked genetic disorder caused by mutations in the gene that encodes the Wiskott-Aldrich syndrome protein (WASp). Here, we report the clinical case of an 18-month-old boy diagnosed with Wiskott-Aldrich syndrome, who did not have an HLA-matched related or unrelated donor and was treated successfully with a hematopoietic stem cell transplant (HSCT) from a haploidentical family donor. Graft-versus-host disease (GvHD) prophylaxis included post-transplant cyclophosphamide (PT-Cy). At day +30, the peripheral blood-nucleated cell chimerism was 100% and the WAS protein had a normal expression. Currently, at month 32 post-transplant, the patient has hematological and immune reconstitution and complete donor chimerism without evidence of GvHD. HSCT with PT-Cy was a feasible and safe option for this patient with WAS, in which an HLA matched donor was not available.

Tumor metastásico en tejido blando a nivel del sacro secundario a carcinoma de células claras de riñón. Reporte de caso / Metastatic tumor in soft tissue at the level of the sacrum secondary toclear cell carcinoma of the kidney. A case report

Introducción. El cáncer de riñón es la undécima neoplasia maligna más común en los Estados Unidos Mexicanos. El carcinoma de células claras de riñón (CCR) es considerado la estirpe más frecuente y representa el 2-3 % de todos los cánceres a nivel mundial. En el contexto de la enfermedad metastásica, por lo general se identifica un tumor renal primario y las metástasis se localizan en pulmón, hueso, hígado, cerebro y, raramente, en tejidos blandos. Los pacientes con metástasis a tejidos blandos no tienen síntomas en las etapas iniciales y generalmente se identifican sólo cuando las lesiones aumentan de tamaño o durante el estudio de la pieza de resección quirúrgica. Caso clínico. Se presenta el caso de una paciente en la séptima década de la vida, con una metástasis en tejidos blandos de la región sacra, de 10 años de evolución posterior a una nefrectomía secundario a CCR. Resultados. Hallazgos clínicos e imagenológicos de un tumor bien delimitado. Se realizó resección quirúrgica de la lesión, bajo anestesia regional, con extirpación completa. Conclusión. Se recomienda que los pacientes con un sitio metastásico resecable y solitario sean llevados a resección quirúrgica con márgenes libres, como fue el caso de nuestra paciente, por su fácil acceso y ser una lesión única. En el CCR, además de su tratamiento quirúrgico inicial, es indispensable una estrecha vigilancia con examen físico e imágenes transversales, para detectar la presencia de metástasis y con ello evitar tratamientos tardíos.

Introduction. Kidney cancer is the eleventh most common malignancy in the United States of Mexico. Carcinoma renal cell (CRC) is considered the most frequent type and represents 2-3% of all cancers worldwide. In the setting of metastatic disease, a primary renal tumor is usually identified, and metastases are located in the lung, bone, liver, brain, and rarely in soft tissue. Patients with soft tissue metastases do not have symptoms in the initial stages and are generally found only when the lesions increase in size or during the study of the surgical resection piece. Clinical case. In this case, we report a female patient in the seventh decade of life with a soft tissue metastasis located in the sacral region, 10 years after a nephrectomy secondary to CRC. Results. Clinical and radiological findings of a well-defined tumor. Surgical resection of the lesion is performed under regional anesthesia with complete excision. Conclusions. It is recommended that patients with a resectable and solitary metastatic site be candidates for surgical resection with free margins, as was the case with our patient due to its easy access and single lesion. In CRC, in addition to its initial surgical treatment, close surveillance with physical examination and cross-sectional images is essential to monitor the presence of metastases and thus avoid late treatments.

Overexpression SPRY2 Suppresses Esophageal Squamous Cell Carcinoma Progression Via Inactivation of ERK/AKT Signaling / La Sobreexpresión SPRY2 Suprime la Evolución del Carcinoma de Células Escamosas de Esófago Mediante la Inactivación de la Señalización ERK/AKT

SUMMARY: Esophageal cancer is one of the most aggressive gastrointestinal cancers. Invasion and metastasis are the main causes of poor prognosis of esophageal cancer. SPRY2 has been reported to exert promoting effects in human cancers, which controls signal pathways including PI3K/AKT and MAPKs. However, the expression of SPRY2 in esophageal squamous cell carcinoma (ESCC) and its underlying mechanism remain unclear. In the present study, we aimed to investigate the detailed role of SPRY2 in the regulation of cell proliferation, invasion and ERK/AKT signaling pathway in ESCC. It was identified that the expression level of SPRY2 in ESCC was remarkably decreased compared with normal tissues, and it was related to clinicopathologic features and prognosis ESCC patients. The upregulation of SPRY2 expression notably inhibited the proliferation, migration and invasion of Eca-109 cells. In addition, the activity of ERK /AKT signaling was also suppressed by the SPRY2 upregulation in Eca-109 cells. Our study suggests that overexpression of SPRY2 suppress cancer cell proliferation and invasion of by through suppression of the ERK/AKT signaling pathways in ESCC. Therefore, SPRY2 may be a promising prognostic marker and therapeutic target for ESCC.

El cáncer de esófago es uno de los cánceres gastrointestinales más agresivos. La invasión y la metástasis son las principales causas de mal pronóstico del cáncer de esófago. Se ha informado que SPRY2 ejerce efectos promotores en los cánceres humanos, que controla las vías de señales, incluidas PI3K/AKT y MAPK. Sin embargo, la expresión de SPRY2 en el carcinoma de células escamosas de esófago (ESCC) y su mecanismo subyacente aún no están claros. En el presente estudio, nuestro objetivo fue investigar el papel detallado de SPRY2 en la regulación de la proliferación celular, la invasión y la vía de señalización ERK/AKT en ESCC. Se identificó que el nivel de expresión de SPRY2 en ESCC estaba notablemente disminuido en comparación con los tejidos normales, y estaba relacionado con las características clínico-patológicas y el pronóstico de los pacientes con ESCC. La regulación positiva de la expresión de SPRY2 inhibió notablemente la proliferación, migración e invasión de células Eca-109. Además, la actividad de la señalización de ERK/AKT también fue suprimida por la regulación positiva de SPRY2 en las células Eca-109. Nuestro estudio sugiere que la sobreexpresión de SPRY2 suprime la proliferación y la invasión de células cancerosas mediante la supresión de las vías de señalización ERK/AKT en ESCC. Por lo tanto, SPRY2 puede ser un marcador de pronóstico prometedor y un objetivo terapéutico para la ESCC.

Avaliação de incapacidade em pessoas com doença falciforme / Disability assessment in people with sickle cell / Evaluación de la discapacidad en personas con enfermedad de células falciformes

A expectativa de vida das pessoas com doença falciforme (DF) é baixa, e o agravamento da condição de saúde é frequente, gerando incapacidades. Todavia, pouco é conhecido sobre tais incapacidades com base na Classificação Internacional de Funcionalidade, Incapacidade e Saúde (CIF). Assim, o objetivo do estudo foi descrever as incapacidades de adultos com DF. Realizou-se um estudo transversal descritivo, com 60 adultos com DF, de ambos os sexos. Informações sociodemográficas, fatores clínicos relacionados ao tipo de DF foram autorreferidos pelos participantes. As incapacidades foram avaliadas por meio da Escala de Avaliação de Incapacidade da Organização Mundial da Saúde (WHODAS 2.0) nos seguintes domínios: cognição, mobilidade, autocuidado, relações interpessoais (convivência com as pessoas), atividades de vida e participação na sociedade. Em cada domínio, foi definida incapacidade quando o sujeito relatou dificuldade leve, moderada, grave ou extrema de desempenhar a tarefa. Os dados foram analisados por estatística descritiva (frequências relativas). Os resultados mostraram valores elevados em todos os domínios avaliados, com destaque para dificuldades de concentração (90%) e desempenho nas tarefas domésticas (93,3%) e impacto da situação de saúde nas finanças do sujeito/família (96,7%). Os dados deste estudo mostraram que adultos com DF apresentam elevados índices de incapacidade, especialmente no domínio cognitivo, nas atividades de vida e na participação social.

Life expectancy of people living with sickle cell disease (SCD) is in general low and the worsening of their health condition is frequent, resulting in disabilities. However, knowledge about those disabilities based on the International Classification of Functioning, Disability and Health (ICF) is scarce. Thus, this study aimed to describe the disabilities of adults with SCD. A descriptive cross-sectional study was carried out with 60 adults with SCD of both sexes. Sociodemographic and clinical factors related to the type of SCD were self-reported by the participants. Disabilities were assessed by the World Health Organization Disability Assessment Schedule (WHODAS 2.0), in the following domains: cognition, mobility, selfcare, getting along with people, life activities, and participation in society. In each domain, disability was defined when the participant self-reported light, moderate, severe, extreme difficulty, or inability to perform the task. Data analysis included descriptive statistics (relative frequencies). The results showed high values in all domains evaluated, with emphasis on difficulties on concentrating (90%) and performing household chores (93.3%) and on the impact of health condition on subject/family's financial situation (96.7%). The data from this study shows that adults with SCD present high disability levels, especially on the cognitive domain, on life activities, and on social participation.

Las personas con enfermedad de células falciformes (ECF) tienen una baja esperanza de vida, y el empeoramiento de su estado de salud es frecuente, generando discapacidades. Sin embargo, estas discapacidades son poco conocidas según la Clasificación Internacional del Funcionamiento, de la Discapacidad y de la Salud (CIF). El objetivo de este estudio fue describir las discapacidades de adultos con ECF . Se realizó un estudio transversal, descriptivo, con 60 adultos con ECF, de ambos sexos. Los factores sociodemográficos y clínicos relacionados con el tipo de ECF fueron autoinformados. Las discapacidades se evaluaron mediante la Escala de Evaluación de la Discapacidad de la Organización Mundial de la Salud (WHODAS 2.0), en los dominios de cognición, movilidad, autocuidado, convivencia con personas, actividades de la vida y participación en la sociedad. La discapacidad se definió mediante un reporte de dificultad o incapacidad leve, moderada, grave, extrema del individuo para realizar una tarea. Los datos fueron analizados por estadística descriptiva (frecuencias relativas). Los resultados mostraron altos valores de discapacidad en todos los dominios evaluados, con énfasis en las dificultades de concentración (90%), el desempeño en las tareas domésticas (93,3%) y el impacto de la situación de salud en las finanzas del sujeto/familia (96,7%). Los datos de este estudio mostraron que los adultos con ECF tienen altas tasas de discapacidad, especialmente en los dominios cognitivo, actividades de la vida y participación social.