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Introducción. Los quistes esplénicos son entidades anatómicas y clínicas poco frecuentes, con una incidencia aproximada de 0,07 %. Se clasifican como quistes esplénicos primarios, que contienen revestimiento epitelial y se subdividen en parasitarios y no parasitarios según su etiología, y quistes secundarios, que no poseen revestimiento epitelial en la luz quística y suelen ser el resultado de un traumatismo abdominal. Por lo general, son asintomáticos y se pueden encontrar de manera incidental durante estudios de imagen o en cirugía. Los síntomas están relacionados con el tamaño de los quistes. El tratamiento ideal es la resección quirúrgica, que puede ser total o parcial. Caso clínico. Paciente femenina de 23 años, sin antecedentes de trauma, con dolor abdominal intermitente, de varios meses de evolución. En los estudios imagenológicos se identificó un pseudoquiste esplénico gigante. Fue tratada mediante esplenectomía total por laparoscopia, sin complicaciones quirúrgicas. Resultados. Tuvo una adecuada evolución postoperatoria. Conclusión.El diagnóstico de quiste esplénico se realiza mediante estudios imagenológicos y se confirma con el análisis histopatológico. La esplenectomía total ha sido el tratamiento tradicional; sin embargo, ahora mediante la implementación de abordajes mínimamente invasivos, se prefiere la esplenectomía parcial, con el fin de preservar tejido esplénico y su función inmunológica.

Introduction.Splenic cysts are rare anatomical and clinical entities, with an approximate incidence of 0.07%. They are classified as primary splenic cysts, which contain epithelial lining and are subdivided into parasitic and non-parasitic depending on their etiology, and the secondary splenic cysts, which do not have an epithelial lining in the cystic lumen and are usually the result of abdominal trauma. They are usually asymptomatic and can be found incidentally during imaging studies or in surgery. The symptoms are related to the size of the cysts. The ideal treatment is surgical resection, which can be total or partial. Clinical case. A 23-year-old female patient, with no history of trauma, with intermittent abdominal pain, lasting several months. Imaging studies identified a giant splenic pseudocyst. She was treated by total laparoscopic splenectomy, without surgical complications. Results. She had an adequate postoperative evolution. Conclusion. The diagnosis of splenic cyst is made through imaging studies and confirmed with histopathological analysis. Total splenectomy has been the traditional treatment; however, now through the implementation of minimally invasive approaches, partial splenectomy is preferred, in order to preserve splenic tissue and its immunological function.

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PURPOSE: To assess liver and spleen characteristics of a population with Gaucher disease (GD) using multiparametric MRI and MR elastography (MRE) for evaluation of diffuse liver and spleen disease, which includes liver fat fraction, liver and spleen volume and iron deposition, and liver and spleen stiffness correlated with DS3 Severity Scoring System for Gaucher disease (GD-DS3). METHODS: We prospectively evaluated 41 patients with type 1 Gaucher disease using a 3.0 T MRI and MRE between January 2019 and February 2020. Clinical, laboratory, and imaging data was collected. Mann-Whitney, Kruskal-Wallis, and Spearman's correlation were applied to evaluate liver and spleen MRI and MRE, clinical and laboratory variables, and GD-DS3. ERT and SRT treatment groups were compared. RESULTS: Hepatomegaly was seen in 15% and splenomegaly in 42% of the population. Moderate and strong and correlations were found between liver and spleen iron overload (rho = 0.537; p = 0.002); between liver and spleen volume (rho = 0.692, p < 0.001) and between liver and spleen stiffness (rho = 0.453, p = 0.006). Moderate correlations were found between liver stiffness and GD-DS3 (rho = 0.559; p < 0.001) and between splenic volume and GD-DS3 (rho = 0.524; p = 0.001). CONCLUSION: The prevalence of hepatosplenomegaly, liver fibrosis, and liver iron overload in treated patients with GD is low, which may be related to the beneficial effect of treatment. Liver MRE and splenic volume correlate with severity score and may be biomarkers of disease severity.

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Background: Dengue fever is a mosquito-borne infectious disease endemic in over 100 countries around the world. Among the complications that dengue can cause the Hemophagocytic Lymphohistiocytosis is one of great concern for its severity and complex diagnosis. Case report: Hereby we document a case of this disease expressed on a previously healthy 6-year-old female patient whose dengue infection was so severe that needed intensive care management with vasoactive drugs and diuretics. After a short period of wellness began newly with fever, pancytopenia, hepatitis, and inflammatory response symptoms. Conclusions: A Dengue associated Hemophagocytic Lymphohistiocytosis syndrome was suspected and treated with intravenous corticosteroids on a 3-day scheme at no signs of malignancy with excellent response. The health care professionals must know about this not novel entity in order to reach an efficient diagnosis and treatment mostly, but not only, those in tropical and sub-tropical regions of the word were dengue virus is endemic.

Antecedentes: La fiebre por dengue es una enfermedad infecciosa transmitida por mosquitos, endémica en más de 100 países alrededor del mundo. La Linfohistiocitosis Hemofagocítica, dentro de las complicaciones que puede ocasionar el dengue, es una de las más preocupantes por su complejidad diagnostica y gravedad. Reporte de caso: Femenino de 6 años de edad, previamente sana, cuya infección por dengue fue tan grave que requirió manejo en cuidados intensivos. Después de un breve período de bienestar recrudeció la fiebre, además de pancitopenia, hepatitis y síntomas de respuesta inflamatoria. Conclusiones: Se sospechó síndrome de Linfohistiocitosis Hemofagocítica asociada a Dengue y se trató con corticoides intravenosos en un esquema de 3 días con excelente respuesta. Los profesionales de la salud deben conocer esta entidad no novedosa para poder llegar a un diagnóstico y tratamiento eficaz en su mayoría, pero no solo, en las regiones tropicales y subtropicales del mundo donde el virus del dengue es endémico.

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Hyper-reactive malarial splenomegaly (HMS), or tropical splenomegaly syndrome, is a severe complication of chronic and recurrent infections caused by Plasmodium spp. This condition typically results in splenomegaly greater than or equal to 10 cm and a constellation of laboratory findings, including the absence of identifiable parasites in peripheral blood smears. However, patients with HMS demonstrate serological or molecular evidence of infection. Despite being a familiar entity in malaria holoendemic countries in Africa, and regions of Papua New Guinea, the pathophysiology, natural history, and treatment of the syndrome remains to be fully elucidated. Herein, we describe a highly suggestive case of HMS in a Senegalese patient migrating northbound to reach the U.S.-Mexico border and for whom we provided medical care during his crossing of the Darien Gap in Panama. We also reviewed the literature on diagnosing and treating HMS in-depth.

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El absceso esplénico es una entidad poco común. Se define como la presencia de una lesión ocupante de espacio en el bazo asociada a infección. Ha sido descrito en 0,14-0,7 % de los estudios de autopsias. Se presenta el caso de un paciente de 60 años, de color de piel blanca, que acudió al hospital por presentar fiebre y diarreas, con dolor en fosa lumbar izquierda. Fue ingresado y trasladado a Unidad de Cuidados Intensivos debido a fallo renal agudo por deshidratación. Al trasladarse a sala abierta se diagnosticó un absceso esplénico mediante ultrasonido y tomografía. No se decidió operar en ese momento porque no tenía criterio. Posteriormente sufrió de abdomen agudo y shock séptico por ruptura del absceso hacia cavidad abdominal y fue intervenido de urgencia; se realizó esplenectomía y toilette de la cavidad. Su evolución posterior fue favorable. Por lo poco común de esta entidad, se decidió la publicación del caso.

The splenic abscess is a rare entity. It is defined as the presence of a space-occupying lesion in the spleen associated with infection. It has been described in 0.14-0.7% of autopsy studies. The case of a 60-years-old patient, white-skinned, who came to the hospital due to fever and diarrhea, with pain in the left lumbar fossa is presented. He was admitted and transferred to the Intensive Care Unit due to acute renal failure due to dehydration. When transferred to the open room, a splenic abscess was diagnosed by ultrasound and tomography. It was not decided to operate at that time because there are no criteria. Subsequently, he presented acute abdomen and septic shock due to rupture of the abscess into the abdominal cavity and underwent emergency surgery, performing splenectomy and the cavity's toilette. His subsequent evolution was positive. Due to the unusual nature of this entity, it was decided to publish the case.

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Background: Predicting the roughly 50% of melanoma patients that will respond to immunotherapy is challenging. We tested if splenic volume could be a predictive biomarker. Methods: Splenic volume was measured by a semiautomated commercial software tool in pre- and post-treatment PET/CT, CT or MRI in 50 melanoma patients treated with immune checkpoint inhibitors. Results: Subjects with smaller spleens had better progression-free survival (median not achieved after 30.6 months of follow-up vs median 11.2 months; p = 0.0213) than their counterparts. A cut-off of <244 cm3 yielded a sensitivity of 83% and specificity of 54% to identify responders. Conclusion: Measuring splenic volume on imaging scans is feasible. Smaller pretreatment spleen volume is associated with better responses to immune checkpoint inhibitors.

For patients with relapsed or advanced melanoma, immunotherapy is the main treatment option. Not all patients respond to it and there are few ways of knowing the odds beforehand. Treatment can be costly and dangerous. We investigated if measuring the spleen using imaging scans already routinely done to monitor the disease could give doctors an idea of whether the patient had higher chances of responding to immunotherapy. Our main finding was that patients with smaller spleens before treatment initiation were more likely to respond to immunotherapy and live longer without the disease. This finding can potentially be used in day-to-day care to inform patients and their physicians of the patient's odds and help them make an informed joint decision.

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Se describe el caso clínico de un adulto joven de 30 años de edad, con antecedente de buena salud, quien fue asistido en el Servicio de Cirugía del Hospital Universitario Manuel Ascunce Domenech en Camagüey por presentar aumento de volumen en la región abdominal de dos meses de evolución, concomitante con astenia, anorexia, vómitos y pérdida de peso. En el examen físico resultaron notables el abdomen globuloso (bazo palpable) sin dolor, un hematoma periumbilical y múltiples adenopatías supraclaviculares; en tanto, en los estudios imagenológicos se evidenció la presencia de esplenomegalia. Se realizó esplenectomía con administración de anestesia general endotraqueal y en el estudio histopatológico de la muestra resecada se confirmó el diagnóstico de linfoma primario del bazo.

The case report of a 30-year-old young adult with a history of good health is described, who was assisted at the Surgery Service of Manuel Ascunce Domenech University Hospital in Camagüey due to volume increase in the abdominal region with a course of two months, concomitant with asthenia, anorexia, vomits and weight loss. Physical examination revealed a globular abdomen (palpable spleen) with no pain, a periumbilical hematoma, and multiple supraclavicular adenopathies; meanwhile, in the imaging studies splenomegaly was evidenced. Splenectomy was performed with administration of general endotracheal anesthesia and the histopathological study of the resected sample confirmed the diagnosis of primary spleenic lymphoma.

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The spleen is supplied by blood flow through the splenic artery and vein. The purpose of this communication is to report an ectopic spleen supplied only by reverse flow through the left gastro-omental vessels. A 14-year-old boy presented with pelvic splenomegaly supplied only by the left gastro-omental artery and veins connected to the inferior polar vessels, which were the only vessels communicating with the spleen. After detorsion of the spleen and splenopexy, the spleen returned to normal dimensions. The patient had uneventful follow-up. In conclusion, the left gastroepiploic vessels are able to maintain the entire spleen blood supply.

O baço é suprido pelo fluxo sanguíneo da artéria e veia esplênicas. O objetivo desta comunicação é apresentar um baço ectópico suprido apenas pelo fluxo sanguíneo reverso proveniente dos vasos gastromentais esquerdos. Um paciente de 14 anos apresentou esplenomegalia pélvica suprida apenas por artéria e veia gastromentais esquerdas, conectadas aos vasos polares inferiores, que eram os únicos presentes nesse baço. Após a distorção do baço e a esplenopexia, o baço voltou às dimensões normais. Não houve intercorrências no acompanhamento do paciente. Em conclusão, os vasos gastromentais esquerdos são capazes de suprir o fluxo sanguíneo de todo o baço.

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Myelofibrosis (MF) is a BCR-ABL1-negative myeloproliferative neoplasm characterized by clonal myeloproliferation, dysregulated kinase signaling, and release of abnormal cytokines. In recent years, important progress has been made in the knowledge of the molecular biology and the prognostic assessment of MF. Conventional treatment has limited impact on the patients' survival; it includes a wait-and-see approach for asymptomatic patients, erythropoiesis-stimulating agents, androgens, or immunomodulatory agents for anemia, cytoreductive drugs such as hydroxyurea for the splenomegaly and constitutional symptoms, and splenectomy or radiotherapy in selected patients. The discovery of the Janus kinase (JAK) 2 mutation triggered the development of molecular targeted therapy of MF. The JAK inhibitors are effective in both JAK2-positive and JAK2-negative MF; one of them, ruxolitinib, is the current best available therapy for MF splenomegaly and constitutional symptoms. Although ruxolitinib has changed the therapeutic scenario of MF, there is no clear indication of a disease-modifying effect. Allogeneic stem cell transplantation remains the only curative therapy of MF, but due to its associated morbidity and mortality, it is usually restricted to eligible high- and intermediate-2-risk MF patients. To improve current therapeutic results, the combination of JAK inhibitors with other agents is currently being tested, and newer drugs are being investigated.

La mielofibrosis (MF) es una neoplasia mieloproliferativa negativa para BCR-ABL1 caracterizada por mieloproliferación clonal, señalización de cinasa desregulada y liberación de citocinas anormales. En los últimos años se han realizado importantes avances en el conocimiento de la biología molecular y la valoración pronóstica de la MF. El tratamiento convencional tiene un impacto limitado en la supervivencia de los pacientes; incluye un enfoque de espera para pacientes asintomáticos, agentes estimulantes de la eritropoyesis, andrógenos o agentes inmunomoduladores para la anemia, fármacos citorreductores como la hidroxiurea para la esplenomegalia y los síntomas constitucionales, y esplenectomía o radioterapia en pacientes seleccionados. El descubrimiento de la mutación Janus cinasa (JAK) 2 desencadenó el desarrollo de la terapia dirigida molecular de la MF. Los inhibidores de JAK son efectivos tanto en MF con JAK2 positivo como con JAK2 negativo; uno de ellos, el ruxolitinib, es la mejor terapia disponible actualmente para la esplenomegalia y los síntomas constitucionales de la MF. Sin embargo, aunque el ruxolitinib ha cambiado el escenario terapéutico de la MF, no hay indicios claros de un efecto modificador de la enfermedad. El alotrasplante de células madre sigue siendo la única terapia curativa de la MF, pero debido a su morbilidad y mortalidad asociadas, generalmente se restringe a pacientes elegibles con MF de riesgo alto e intermedio 2. Para mejorar los resultados terapéuticos actuales, actualmente se está probando la combinación de inhibidores de JAK con otros agentes y se están investigando fármacos más nuevos.

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Abstract The spleen is supplied by blood flow through the splenic artery and vein. The purpose of this communication is to report an ectopic spleen supplied only by reverse flow through the left gastro-omental vessels. A 14-year-old boy presented with pelvic splenomegaly supplied only by the left gastro-omental artery and veins connected to the inferior polar vessels, which were the only vessels communicating with the spleen. After detorsion of the spleen and splenopexy, the spleen returned to normal dimensions. The patient had uneventful follow-up. In conclusion, the left gastroepiploic vessels are able to maintain the entire spleen blood supply.

Resumo O baço é suprido pelo fluxo sanguíneo da artéria e veia esplênicas. O objetivo desta comunicação é apresentar um baço ectópico suprido apenas pelo fluxo sanguíneo reverso proveniente dos vasos gastromentais esquerdos. Um paciente de 14 anos apresentou esplenomegalia pélvica suprida apenas por artéria e veia gastromentais esquerdas, conectadas aos vasos polares inferiores, que eram os únicos presentes nesse baço. Após a distorção do baço e a esplenopexia, o baço voltou às dimensões normais. Não houve intercorrências no acompanhamento do paciente. Em conclusão, os vasos gastromentais esquerdos são capazes de suprir o fluxo sanguíneo de todo o baço.

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Primary splenic angiosarcoma is a malignant vascular neoplasm with a short-term fatal prognosis in most cases, with nonspecific symptoms and usually in advanced stages. We present the case of a 49-year-old man with clinical history of chronic diarrhea and weight loss, in which it was identifies splenomegaly. The histopathological study disclose the diagnosis of primary angiosarcoma of the spleen, with spread to the liver, lung, and lymph nodes. The patient was discharged after undergoing splenectomy, with palliative treatment. Because the rarity of this neoplasm, there is limited experience regarding the best management with a significant impact on patient survival.

El angiosarcoma esplénico primario es una neoplasia vascular maligna con un pronóstico mortal a corto plazo en la mayoría de los casos, que se presenta con síntomas inespecíficos y usualmente en estadios avanzados. Presentamos el caso de un hombre de 49 años, con historia de diarrea crónica y pérdida de peso, en el que se identificó esplenomegalia. El estudio patológico reveló el diagnóstico de angiosarcoma primario de bazo, con diseminación en hígado, pulmón y ganglios linfáticos. Debido a la rareza de esta neoplasia existe poca experiencia en relación con el mejor manejo con impacto significativo en la supervivencia de los pacientes.

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RESUMEN La enfermedad de Gaucher es la enfermedad más frecuente del grupo de las enfermedades de depósito lisosomal, la cual pertenece a los errores innatos del metabolismo. Se produce acumulación de glucocerebrósidos en diferentes órganos y como consecuencia, el desarrollo de síntomas y signos multisistémicos que se establecen de manera crónica y progresiva, tales como: visceromegalias, destrucción ósea y citopenias periféricas. Se presenta un paciente masculino de 43 años con diagnóstico de esta enfermedad desde la infancia, que desarrolló esplenomegalia gigante con hiperesplenismo, por lo que requirió esplenectomía, obteniéndose buenos resultados con el tratamiento quirúrgico.

ABSTRACT Gaucher disease is the most common disease of the lysosomal storage diseases group comprised within innate errors of metabolism. There is glucocerebrosides accumulation in different organs and as a consequence, the development of symptoms and multisystemic signs that are established in a chronic and progressive way, such as: visceromegaly, bone destruction and peripheral cytopenias. A 43-years-old male patient is presented with a diagnosis of this disease since childhood, who developed giant splenomegaly with hypersplenism, for which he required splenectomy, obtaining good results with surgical treatment.

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O angiossarcoma primário hepático é o tumor mesenquimal mais comum do fígado, representando cerca de 2% das neoplasias malignas primárias do órgão. Esse raro tumor tem sintomas inespecíficos, evolução agressiva e diagnóstico usualmente tardio, com prognóstico reservado mesmo quando tratado. Este trabalho consiste em um relato de caso de um paciente do sexo masculino, de 44 anos, que foi encaminhado à emergência do Hospital Geral Roberto Santos para investigação de quadro de anemia grave sintomática, síndrome consumptiva e hepatoesplenomegalia. Durante investigação laboratorial, evidenciou-se anemia com provável componente microangiopático associado à anemia da doença crônica. As sorologias para doenças virais e baciloscopia do escarro foram negativas. Foram detectados em exames de imagem dois nódulos hepáticos de grandes dimensões, adenomegalias retroperitonais, esplenomegalia de grande monta, volumoso derrame pleural à direita, além de alterações do esqueleto axial e apendicular. Evoluiu com síndrome da lise tumoral após tratamento clínico com corticoterapia por suspeita de linfoma, com óbito. A biópsia guiada por uma tomografia realizada previamente teve como conclusão perfil imuno-histoquímico compatível com angiossarcoma hepático. O angiossarcoma é um raro tumor, de difícil diagnóstico e tratamento, com evolução agressiva e achados clínico-laboratoriais pouco elucidativos, devendo a hipótese desta doença ser considerada no diagnóstico diferencial das neoplasias hepáticas. As opções terapêuticas são limitadas. Relatos de casos como este são de suma importância para o aumento do grau de suspeição clínica e um diagnóstico mais precoce dessa entidade de costumeira evolução catastrófica.

Primary hepatic angiosarcoma is the most common mesenchymal tumor of the liver, representing about 2% of primary hepatic malignancies. This rare tumor has nonspecific symptoms, delayed diagnosis, and aggressive evolution, with a poor prognosis even when treated. This study reports the case of a 44-year-old male patient referred to the emergency department of the Hospital Geral Roberto Santos with symptomatic severe anemia, consumptive syndrome, and hepatosplenomegaly. Laboratory investigation indicated anemia with a probable microangiopathic component and chronic disease anemia. Serology tests for viral diseases returned negative results, as well as sputum smear microscopy for tuberculosis. Imaging exams revealed two large hepatic nodules, retroperitoneal adenomegaly, large splenomegaly, large pleural effusion in the right lung, and bone involvement. After clinical treatment with corticosteroids for suspected lymphoma, the patient evolved with tumor lysis syndrome and died. Tomography-guided liver biopsy was previously performed, indicating an immunohistochemical profile compatible with hepatic angiosarcoma ­ a rare tumor of difficult diagnosis and treatments due to its aggressive evolution and poor clinical and laboratory findings. Considering the nonspecificity of imaging exams, this disease should be considered in the differential diagnosis of liver neoplasms investigation. Case reports such as the one described in this study are important for increasing the degree of clinical suspicion and earlier diagnosis of this malignancy.

El angiosarcoma hepático primario es el tumor mesenquimatoso del hígado más común y representa el 2% de las neoplasias malignas primarias del hígado. Este raro tumor presenta una sintomatología inespecífica, diagnóstico tardío y evolución agresiva, con mal pronóstico incluso en tratamiento. Este es un reporte de caso de un hombre de 44 años de edad, que fue remitido al servicio de urgencias del Hospital Geral Roberto Santos para investigar anemia severa sintomática, síndrome de consunción y hepatoesplenomegalia. Durante la investigación de laboratorio, se evidenció anemia con un probable componente microangiopático asociado a anemia por enfermedad crónica. La serología para enfermedades virales resultó negativa, así como la microscopía de frotis de esputo para tuberculosis. Las imágenes revelaron dos grandes nódulos hepáticos, adenomegalia retroperitoneal, gran esplenomegalia, gran derrame pleural en el pulmón derecho, así como afectación del esqueleto axial y apendicular. El paciente evolucionó con síndrome de lisis tumoral tras el tratamiento clínico con corticoides por sospecha de linfoma, y no se resistió. Previamente se realizó biopsia hepática guiada por tomografía con perfil inmunohistoquímico compatible con angiosarcoma hepático. El angiosarcoma es un tumor raro, de difícil diagnóstico y tratamiento por su evolución agresiva y deficientes hallazgos clínicos y de laboratorio. Los exámenes por imágenes son inespecíficos y la posibilidad de esta enfermedad debe considerarse en el diagnóstico diferencial de la investigación de neoplasias hepáticas. Las opciones terapéuticas son limitadas. Reportes de casos como este son importantes para incrementar el grado de sospecha clínica y el diagnóstico precoz de este tipo de evolución catastrófica habitual.

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It is well documented that sporadic Alzheimer's disease (AD) is a multifactorial disease and considered to be a result of several pathological events, both in the periphery and in the brain. The role of the peripheral immune system in the etiology and/or progression of the disease is not fully understood yet, and the results in humans are contradictory so far. Several animal models of AD have been generated and thoroughly characterized to elucidate disease mechanisms and evaluate numerous therapeutic strategies in preclinical studies. In the present study, we carried out a longitudinal evaluation of blood lymphocytes from male and female 3xTg-AD mice to document important immunological abnormalities in the periphery. We documented the age-dependent decrease in the percentage of CD3+ and CD4+ lymphocytes and an increase in the percentage CD3+CD4-CD8- (DN T) cells in the blood of 3xTg-AD mice compared with non-transgenic animals. Severe splenomegaly was observed in 3xTg-AD mice in contrast to wild-type animals. Importantly, all these abnormalities in the peripheral immune system appeared earlier and were more pronounced in males compared with females of the same age, which may account for the shorter lifespan of male mice. We suggest that future research should include the measurement of CD3+ and DN T cells as a potential immunological marker of disease progression in AD patients.

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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%.

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Abstract Introduction: Inflammation promotes the progression of chronic renal failure, and the start of dialysis worsens inflammation. The enlargement of the spleen is associated with inflammation, and patients on hemodialysis may show a large spleen. The aim of the present study was to compare the spleen size of patients undergoing hemodialysis versus controls to update this thread. Methods: Controls and patients were eligible to participate in the study provided they were negative for serological markers of hepatitis B and C viruses and HIV, if they had no lymphoproliferative disorder, and if they were at least 18 years of age. Age, sex, and the duration of dialysis were recorded. Laboratory variables (hemoglobin, hematological cell count, serum creatinine) and the underlying cause of end-stage renal disease were analyzed. The spleen sizes of the patients were divided into tertiles. Results: The 75 controls and 168 patients selected were sex-matched. The patients were older, had larger spleens and lower platelet counts than controls. The relationship between spleen size and age in the controls and patients was quite similar. The patients in the first tertile of spleen size compared with those in the third were older and had a higher platelet counts. The underlying disease and dialysis vintage had no effect on spleen size. Discussion: The patients had larger spleens and a greater range of spleen sizes than the controls. In patients, the association between larger and smaller spleen with lower and higher platelet counts, respectively, sparked the speculation of occurrence of hypersplenism and hyposplenism.

Resumo Introdução: A inflamação promove a progressão da insuficiência renal crônica, e o início da diálise agrava a inflamação. O aumento do baço está associado à inflamação e os pacientes em hemodiálise podem apresentar um baço grande. O objetivo do presente estudo foi comparar o tamanho do baço de pacientes em hemodiálise versus aquele de controles, para atualizar este tópico. Métodos: Controles e pacientes foram elegíveis para participar do estudo desde que fossem negativos para marcadores sorológicos dos vírus da hepatite B, C e HIV, se não apresentassem distúrbio linfoproliferativo e tivessem pelo menos 18 anos de idade. Registramos idade, sexo e duração da diálise. Avaliamos as variáveis laboratoriais (hemoglobina, contagem de células hematológicas, creatinina sérica) e a causa básica da doença renal terminal. O tamanho dos baços dos pacientes foram divididos em tercis. Resultados: Os 75 controles e 168 pacientes selecionados foram pareados por sexo. Os pacientes eram mais velhos, tinham baços maiores e menor contagem de plaquetas do que os controles. A relação entre o tamanho do baço e a idade dos controles e pacientes foi bastante semelhante. Os pacientes do primeiro tercil de tamanho do baço, em comparação com os do terceiro, eram mais velhos e apresentavam contagens de plaquetas mais altas. A doença subjacente e o período de diálise não tiveram efeito no tamanho do baço. Discussão: Os pacientes tinham baços maiores e uma maior variedade de tamanhos de baço do que os controles. Entre os pacientes, a associação entre baço maior e menor com contagens de plaquetas mais baixas e mais altas, respectivamente, gerou a especulação da ocorrência de hiperesplenismo e hiposplenismo.

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RESUMEN Se presentó un paciente de 22 años de edad, masculino, de la raza negra con gran esplenomegalia debido a un hemangioma gigante del bazo, el cual presentó trombocitopenia y trastornos de la coagulación. Se estudió y se diagnosticó un síndrome de Kasabach-Merrit. Se le realizó una esplenectomía total anterior regresando a la normalidad el conteo de plaquetas y los trastornos de la coagulación. Su evolución fue favorable al cabo de los 18 meses de seguimiento. Se recomienda el tratamiento quirúrgico, la esplenectomía. Se revisó el tema por lo infrecuente de la enfermedad.

ABSTRACT A 22-year-old male black patient presented with big splenomegaly due to a giant hemangioma of the spleen, showing thrombocytopenia and bleeding disorders. A Kasabach-Merrit syndrome was diagnosed. An anterior total splenectomy was performed, returning the platelet count and coagulation disorders back to normal. His evolution was favorable after 18 months of follow-up. Splenectomy surgical treatment was recommended. The topic was reviewed due to the infrequency of the disease.

RESUMO Foi apresentado paciente do sexo masculino, 22 anos de idade, de raça negra, apresentou grande esplenomegalia devido a hemangioma gigante do baço, que apresentava plaquetopenia e distúrbios de coagulação. Uma síndrome de Kasabach-Merrit foi estudada e diagnosticada. Uma esplenectomia total anterior foi realizada, retornando a contagem de plaquetas e distúrbios de coagulação ao normal. Sua evolução foi favorável após 18 meses de seguimento. Tratamento cirúrgico é recomendado, esplenectomia. O tema foi revisado devido à raridade da doença.

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BACKGROUND AND OBJECTIVES: To determine whether the persistence of splenomegaly characteristic of the Asian haplotype of homozygous sickle cell (SS) disease is associated with continued splenic function, a comparison of patients from Odisha, India, and Jamaica. MATERIALS AND METHODS: Indian patients were examined in a cross-sectional study and compared with the Jamaican Cohort Study from birth. Splenomegaly was assessed in both populations with standard methods. Splenic function was assessed in both by counts of pitted red blood cells determined by differential interference contrast microscopy in the same laboratory. RESULTS: In Jamaica, the spleen became palpable in 55% of patients during the 1st year of life and the prevalence declined thereafter, whereas in Indian patients, the prevalence rose steeply after the age of 4 years. Raised pitted red cell counts, consistent with loss of splenic function, were common after 2 years in Jamaicans but did not increase in Indians until after the age of 5 years. INTERPRETATION AND CONCLUSIONS: The maximal risk of invasive pneumococcal infection in SS disease falls sharply after the age of 3 years, and persistence of splenic function in Odisha patients beyond this age may explain the apparent absence of pneumococcal septicemia in Indian patients and questions the role of pneumococcal prophylaxis.

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Background: Canine visceral leishmaniasis (CVL) is a parasitic disease of high lethality caused by the protozoan Leishmania infantum in Brazil and is often related to splenomegaly. However, splenic nodules in dogs, although frequent, have not previously been reported as associated with CVL, but with neoplastic diseases. Considering that most dogs infected are oligosymptomatic or asymptomatic and that splenic nodules are common to other diseases, it is prudent to differentially diagnose CVL in view of its high zoonotic potential and lethality. The objective of the study was to describe a case of splenomegaly with splenic nodules associated with CVL in an asymptomatic dog treated with 2% miltefosina. Case: A 5-year-old male Rottweiler with 41 kg, with a history of inappetence, apathy and weight loss was referred to the Veterinary Medicine School Clinic of the Cesmac University Center, Maceió, AL, Brazil. However, during palpation a slight increase in the spleen was noted. Hematological, hemoparasite, biochemical and abdominal ultrasonographic examinations were requested to clarify the clinical suspicion of hemoparasitosis. The hematological and biochemical results respectively showed the following: normocytic normochromic anemia, hyperproteinemia and thrombocytopenia, in addition to hypoalbuminemia, with elevated total protein levels. The test for hemoparasites was negative. Ultrasonography showed mixed echogenicity suggestive of nodules. The rapid test for Ehrlichia, Anaplasma and L. infantum was performed. It was positive only for L. infantum. ELISA, IFAT and qPCR tests were performed to confirm the result. The test showed a cutoff result of 0.371 for ELISA, positive for RIFI at a cut-off of 1:40 and qPCR with less than 1 fg and with amplification above 36 cycles. In view of these results, treatment with 2% miltefosine at a dose of 1 mL/ 10 kg was started once a day, after feeding, for 28 days. The animal was monitored throughout treatment and re-evaluated every 10 days for 30 days, showing signs of clinical development, presenting satisfactory results. Discussion: Canine splenomegaly can be associated with a variety of disease possibilities. In asymptomatic canine visceral leishmaniasis (CanL), the slight increase in spleen and the presence of splenic nodules may lead to a false diagnosis. Splenic nodules may be associated with dogs of advanced age and may be due to lymphoid nodular hyperplasia, which causes nodules with echogenicity, hyperechoic regions with well demarcated irregularity, with centralized hypoechoic areas and an absence of hematological and biochemical alterations. The cause of splenomegaly associated with nodules may be difficult to diagnose and require much time and effort. Therefore, diseases such as visceral leishmaniasis of high lethality must be the priority in differential diagnosis in endemic areas in order to minimize the risk of transmission. In addition to allowing an early intervention aiming at good animal health results and preventive measures, such as the use of repellent collars that reduce the risk of phlebotomo infection. The differential diagnosis of CVL is necessary in endemic areas, even in asymptomatic dogs that may present splenic alterations suggestive of other diseases. Treatment with 2% miltefosine was shown to be, in this case, effective at reducing the splenic nodules and a good alternative for the quality of life of the animal.(AU)

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