RESUMO
Background: Hemophagocytic syndrome or hemophagocytic lymphohistiocytosis (HL) is an immune hyperactivation of multifactorial etiology, characterized by excessive activation of lymphocytes and macrophages, as well as numerous pro-inflammatory cytokines. It has a non-specific and highly variable clinical presentation, with splenomegaly being one of the clinical manifestations. Due to its nature, it can manifest during childhood or adult life, which is why it is a disease of diagnostic and therapeutic complexity. Clinical case: 38-year-old male patient without comorbidities, who presented with abdominal pain, choluria, fever > 38 °C and diaphoresis of more than 10 days of evolution. A bone marrow aspirate was performed as part of the diagnostic approach with data compatible with hemophagocytosis and cytopenias. The immunosuppressive management did not show the expected response, which is why an open splenectomy was performed as the last therapeutic option with adequate hematological control. A documentary review of the disease was carried out, and of the therapeutic options, emphasizing surgical management in case of refractoriness to medical treatment. Conclusions: Splenectomy increases the overall survival rate and the time free of HL progression, even though there are still no studies to determine with certainty the ideal time to perform a splenectomy in patients with pancytopenia without splenomegaly who suffer from hemophagocytic syndrome.
Introducción: el síndrome hemofagocítico o linfohistiocitosis hemofagocítica (LH) es una hiperactivación inmune de etiología multifactorial, caracterizada por activación excesiva de linfocitos y macrófagos, así como por numerosas citocinas proinflamatorias. Tiene una presentación clínica poco específica y muy variable, y la esplenomegalia es una de las manifestaciones clínicas. Debido a su naturaleza puede manifestarse durante la infancia o la vida adulta, por lo que es una enfermedad de complejidad diagnóstica y terapéutica. Caso clínico: paciente del sexo masculino de 38 años sin comorbilidades, quien presentó dolor abdominal, coluria, fiebre > 38 °C y diaforesis de más de 10 días de evolución. Se le hizo aspirado de médula ósea como parte del abordaje diagnóstico con datos compatibles con hemofagocitosis y citopenias. El manejo inmunosupresor no mostró la respuesta esperada, por lo que se hizo esplenectomía abierta como última opción terapéutica con adecuado control hematológico. Se hizo una revisión documental de la enfermedad y de las opciones terapéuticas con énfasis en el manejo quirúrgico en caso de refractariedad al tratamiento médico. Conclusiones: la esplenectomía aumenta la tasa de supervivencia general y el tiempo libre de progresión de la LH, aunque no hay todavía estudios para determinar con certeza el tiempo ideal para hacer una esplenectomía en pacientes con pancitopenia sin esplenomegalia que padezcan síndrome hemofagocítico.
Assuntos
Linfo-Histiocitose Hemofagocítica , Esplenectomia , Linfo-Histiocitose Hemofagocítica/cirurgia , Linfo-Histiocitose Hemofagocítica/diagnóstico , Humanos , Masculino , Adulto , Esplenectomia/métodosRESUMO
OBJECTIVE: Several studies have investigated the correlation between the effects of different surgical treatments and laboratory exams for schistosomal portal hypertension, especially concerning portal system thrombosis. The etiopathogenic factors of this thrombosis are not fully understood. In this study, the correlation between surgical treatment for schistosomal portal hypertension and the occurrence of postoperative portal system thrombosis was investigated. METHODS: A total of 61 patients who underwent surgical treatment for schistosomal portal hypertension were distributed into four groups: Patients in Group 1 (n=12) underwent portal variceal disconnection associated with splenic artery ligation and spleen preservation. Patients in Group 2 (n=20) underwent portal variceal disconnection and total splenectomy. Patients in Group 3 (n=20) underwent portal variceal disconnection with subtotal splenectomy, preserving the upper splenic pole supplied by the splenogastric vessels. Patients in Group 4 (n=9) underwent portal variceal disconnection with total splenectomy and autogenous splenic implants on the greater omentum. Late postoperative portal vein thrombosis was diagnosed using Doppler ultrasound. RESULTS: Over the 10-year follow-up, portal vein thrombosis occurred in 26 operated patients (42.6%), with no significant difference observed among the four surgical groups (p=0.217). Most of the thrombi only partially occluded the portal system veins. All the patients presented with a thrombus inside the portal vein. There was no difference in hematological and biochemical tests between groups with or without portal vein thrombosis. CONCLUSIONS: Portal vein thrombosis is often observed in the late postoperative period, irrespective of the surgical treatment employed, and is not associated with patient characteristics or any hematological and biochemical tests.
Assuntos
Hipertensão Portal , Veia Porta , Esplenectomia , Trombose Venosa , Humanos , Hipertensão Portal/cirurgia , Hipertensão Portal/etiologia , Feminino , Masculino , Esplenectomia/efeitos adversos , Esplenectomia/métodos , Veia Porta/cirurgia , Veia Porta/diagnóstico por imagem , Adulto , Pessoa de Meia-Idade , Trombose Venosa/etiologia , Trombose Venosa/cirurgia , Trombose Venosa/diagnóstico por imagem , Resultado do Tratamento , Adulto Jovem , Esquistossomose/cirurgia , Esquistossomose/complicações , Seguimentos , Complicações Pós-Operatórias , Ligadura/métodos , Varizes Esofágicas e Gástricas/cirurgia , Varizes Esofágicas e Gástricas/etiologia , Adolescente , Ultrassonografia DopplerRESUMO
Splenectomy remains an effective treatment for refractory immune cytopenia (RIC), which encompasses immune thrombocytopenia (ITP) and autoimmune hemolytic anemia (AIHA). Accessory spleens (AS) have been described without identifying specific risk factors. We retrospectively analyzed patients with RIC after splenectomy who underwent splenic scintigraphy (SS) at our institution. Seventy-one patients were included. Sixty-two patients had ITP, five had AIHA, and four had Evans syndrome. Seventy-five percent (n = 53) were women. Eleven patients (15.5%) had an AS detected by SS. A complete response (CR) to first-line steroids (odds ratio (OR) 5.75, 95% confidence interval (CI) 1.37-24.14, p = 0.017) and the absence of Howell-Jolly bodies (HJB) in peripheral blood smear (PBS) (OR 11.37, 95% CI 2.70-47.85, p = 0.001) were found to be risk factors. Patients with both elements had a higher rate of AS (83.3%) when compared to those with one or no factors (p < 0.001). Eight patients (73%) underwent an accessory splenectomy: seven (87.5%) achieved a CR, and none had perioperative complications. The presence of HJB in PBS changed from 25 to 87.5% after accessory splenectomy. We recommend the search for an AS via SS in patients with RIC due to ITP, who had a CR to corticosteroids and the absence of HJB in PBS. Accessory splenectomy is a safe and effective procedure.
Assuntos
Púrpura Trombocitopênica Idiopática , Esplenopatias , Trombocitopenia , Humanos , Feminino , Masculino , Estudos Retrospectivos , Esplenectomia/métodos , Trombocitopenia/etiologia , Púrpura Trombocitopênica Idiopática/cirurgia , Púrpura Trombocitopênica Idiopática/etiologia , Esplenopatias/etiologiaRESUMO
Splenectomy is necessary in beta thalassemia major patients when the spleen becomes hyperactive, leading to extreme destruction of erythrocytes. This study assessed the ferritin effect on serum pneumococcal antibody response following pneumococcal vaccination, in patients with beta thalassemia major after splenectomy. In this case series study, convenience sampling was used to recruit 347 splenectomised beta thalassemia patients under the auspices of Jahrom University of Medical Sciences. Demographic data such as age, sex, and time after splenectomy were recorded by a questionnaire. All participants had been splenectomised and received a dose of Pneumovax1 23 vaccine 14 days before surgery. The IgG antibody responses to pneumococcal vaccine and levels of serum specific ferritin were determine by commercial enzyme immunoassay kits. For the analysis, SPSS software version 16 was used. A p-value less than 0.05 was considered statistically significant. Most of the participants (63.4 percent) were hypo-responders to pneumococcal vaccine. Also, serum anti-pneumococcal IgG antibody was related to post splenectomy duration and serum ferritin (p 0.05). An important result was a relation of serum anti-pneumococcal IgG antibody to serum ferritin according to post splenectomy duration groups. Therefore, in three groups of post splenectomy duration, the serum ferritin was higher in hypo-responder than in good responder subjects. Our results indicate that serum anti-pneumococcal IgG antibody decreased with increment of serum ferritin and post splenectomy duration. Thus, there is a need to re-address the approach towards revaccination in this immune-compromised group of patients by administering a booster pneumococcal vaccination in an attempt to recover immunity and reduce morbidity(AU)
La esplenectomía es necesaria en pacientes con beta talasemia mayor cuando el bazo se vuelve hiperactivo, lo que lleva a una destrucción extrema de los eritrocitos. Este estudio evaluó el efecto de la ferritina sobre la respuesta de anticuerpos antineumocócicos en suero después de la vacunación antineumocócica, en pacientes con talasemia beta mayor a los que se les realizó esplenectomía. En este estudio de serie de casos, se utilizó un muestreo de conveniencia para reclutar a 347 pacientes con beta talasemia esplenectomizados bajo los auspicios de la Universidad de Ciencias Médicas de Jahrom. Los datos demográficos como la edad, el sexo y el tiempo después de la esplenectomía se registraron mediante un cuestionario. Todos los participantes fueron esplenectomizados y recibieron una dosis de la vacuna Pneumovax® 23, 14 días antes de la cirugía. Las respuestas de anticuerpos IgG a la vacuna neumocócica y los niveles de ferritina sérica específica se determinaron mediante estuches comerciales de inmunoensayo enzimático. Para el análisis se utilizó el programa SPSS versión 16. Un valor de p inferior a 0,05 se consideró estadísticamente significativo. La mayoría de los participantes (63,4 por ciento) resultaron hiporrespondedores a la vacuna antineumocócica. Además, el anticuerpo sérico antineumocócico IgG se relacionó con la duración de la esplenectomía y la ferritina sérica (p0,05). Un resultado importante fue la relación del anticuerpo sérico IgG antineumocócico con la ferritina sérica según los grupos de duración postesplenectomía. Por lo tanto, en tres grupos de duración posterior a la esplenectomía, la ferritina sérica fue mayor en los sujetos con hiporrespuesta que en los sujetos con buena respuesta. Nuestros resultados indican que el anticuerpo sérico IgG antineumocócico disminuyó con el incremento de la ferritina sérica y la duración posterior a la esplenectomía. Por lo tanto, existe la necesidad de volver a abordar el enfoque hacia la revacunación en este grupo de pacientes inmunocomprometidos mediante la administración de una vacunación antineumocócica de refuerzo en un intento por recuperar la inmunidad y reducir la morbilidad(AU)
Assuntos
Humanos , Masculino , Feminino , Esplenectomia/métodos , Talassemia beta/epidemiologia , Vacinas Pneumocócicas/uso terapêutico , Ferritinas/uso terapêutico , Irã (Geográfico)RESUMO
Introducción: El cierre con pérdida masiva de tejido de la pared abdominal constituye un reto en la cirugía. Objetivo: Presentar un caso de cierre abdominal difícil donde se utilizó la técnica plicatura de la bolsa Bogotá hasta lograr aproximación de los planos cutáneo, muscular y aponeurótico. Caso clínico: Paciente recibido en emergencias con herida perforo-cortante en fosa lumbar izquierda. En la intervención se encontró hemoperitoneo por lesión esplénica, diafragmática y sección del ángulo esplénico del colon. Se le realizó esplenectomía, pleurostomía izquierda y rafia de lesión colónica. Pasadas cinco horas se reintervino por signos de choque y se encontró hematoma retroperitoneal y sección completa del polo superior del riñón izquierdo. Se le realizó nefrectomía izquierda. Al quinto día del posoperatorio mostró signos peritoneales y la exploración confirmó peritonitis fecaloidea por dehiscencia de sutura de colon. Se lavó cavidad y se le ejecutó colostomía tipo Devine. La pérdida masiva de pared abdominal obligó a emplear una plicatura de la bolsa Bogotá para aproximar ambos colgajos, se obtuvo aproximación paulatina hasta la síntesis de la pared. Conclusiones: El abdomen catastrófico es una condición grave y de difícil tratamiento. La bolsa Bogotá es una alternativa viable para esta condición y modificada con plicaturas paulatinas permite el acercamiento de los dos colgajos cutáneos y garantiza el cierre de la pared abdominal, además de ser una técnica fácil de usar y económica(AU)
Introduction: Closure with massive loss of abdominal wall tissue is a challenge in surgery. Objective: To report a case of difficult abdominal closure where the Bogotá bag technique was used until the approximation of the cutaneous, muscular and aponeurotic planes was achieved. Clinical case report: This patient was received in the emergency room with a perforating-cutting wound in the left lumbar fossa. In the intervention, hemoperitoneum was found due to splenic and diaphragmatic injury and section of the splenic flexure of the colon. Splenectomy, left pleurostomy and colonic injury raffia were performed. After five hours, the patient was operated again due to signs of shock. We found a retroperitoneal hematoma and a complete section of the upper pole of the left kidney. It was decided to perform left nephrectomy. On the fifth postoperative day, this patient showed peritoneal signs and examination confirmed fecal peritonitis due to dehiscence of the colon suture. Cavity was washed and a Devine-type colostomy was performed. The massive loss of the abdominal wall made it necessary to use a plication of the Bogotá bag to approximate both flaps, a gradual approximation was obtained until the synthesis of the wall. Conclusions: The catastrophic abdomen is a serious condition that is difficult to treat. The Bogotá bag is a viable alternative for this condition and modified with gradual plications, it allows the two skin flaps to approach each other and guarantees the closure of the abdominal wall, in addition to being an easy-to-use and economical technique(AU)
Assuntos
Humanos , Masculino , Pessoa de Meia-Idade , Colostomia/métodos , Parede Abdominal/cirurgia , Colo Transverso/cirurgia , Serviço Hospitalar de Emergência , Esplenectomia/métodos , Relatório de Pesquisa , Nefrectomia/efeitos adversosRESUMO
BACKGROUND: There are some difficulties regarding the evaluation of the post-splenectomy state. OBJECTIVE: The objective of the study is to compare the post-splenectomy blood changes of immune thrombocytopenia (ITP) patients with those of trauma patients, 1 month and ≥ 6 months after surgery. METHODS: Medical records of patients, who had undergone total splenectomy for ITP and trauma at a tertiary center between January 2009 and December 2019, were retrospectively reviewed. RESULTS: The current study included 52 patients, who had undergone splenectomy for ITP (57.7%), and trauma (42.3%). Splenectomy, irrespective of the indications, resulted in an increase in hemoglobin concentration, hematocrit, and platelet levels. Neutrophils were responsible for the preoperative leukocytosis in ITP patients, and neutrophilia was ameliorated by splenectomy and also withdrawal of the steroid therapy in some patients. Decreased neutrophil-to-lymphocyte ratio and platelet-to-lymphocyte ratio supported the finding that splenectomy ameliorated inflammation in ITP patients. Splenectomy resulted in a change in percentages of leukocytes in favor of basophils in ITP patients. CONCLUSIONS: Splenectomy, irrespective of the indications, resulted in an increase in hemoglobin concentration, hematocrit and platelet levels, lymphocyte, monocyte, and eosinophil counts. Splenectomy ameliorated inflammation in ITP patients and resulted in a change in percentages of leukocytes in favor of basophils.
ANTECEDENTES: Existen algunas dificultades con respecto a la evaluación del estado post-esplenectomía. OBJETIVO: Comparar los cambios sanguíneos post-esplenectomía de pacientes con PTI con los de pacientes traumatizados, 1 mes y ≥ 6 meses después de la cirugía. MÉTODOS: Se revisaron retrospectivamente las historias clínicas de los pacientes que habían sido sometidos a esplenectomía total por PTI y trauma en un centro terciario entre enero de 2009 y diciembre de 2019. RESULTADOS: El presente estudio incluyó a 52 pacientes, que habían sido sometidos a esplenectomía por PTI (57.7%) y traumatismo (42.3%). La esplenectomía, independientemente de las indicaciones, resultó en un aumento de la concentración de hemoglobina, hematocrito y niveles de plaquetas. Los neutrófilos fueron responsables de la leucocitosis preoperatoria en pacientes con PTI, y la neutrofilia mejoró mediante esplenectomía y también la suspensión de la terapia con esteroides en algunos pacientes. La disminución de NLR y PLR apoyó el hallazgo de una disminución de la inflamación en la esplenectomía en pacientes con PTI. La esplenectomía resultó en un cambio en los porcentajes de leucocitos a favor de los basófilos en pacientes con PTI. CONCLUSIONES: La esplenectomía, independientemente de las indicaciones, resultó en un aumento de la concentración de hemoglobina, niveles de hematocrito y plaquetas, recuentos de linfocitos, monocitos y eosinófilos. Una disminución de la inflamación en la esplenectomía en pacientes con PTI resultó en un cambio en los porcentajes de leucocitos a favor de los basófilos.
Assuntos
Púrpura Trombocitopênica Idiopática , Plaquetas , Humanos , Contagem de Plaquetas , Púrpura Trombocitopênica Idiopática/cirurgia , Estudos Retrospectivos , Esplenectomia/métodos , Resultado do TratamentoRESUMO
INTRODUCTION: several pancreatectomy techniques in rats have been described and utilized for research concerning the pancreas endocrine and exocrine functions. However, we did not find a description of any kind of laparoscopic pancreatectomy in rats in the consulted databases. The objective of this study is to describe a laparoscopic splenic lobe pancreatectomy in rats. METHODS: ten Wistars rats weighting more then 300 g were operated for standardizing the laparoscopic pancreatosplenectomy technique, aided by previous descriptions of laparoscopic splenectomy and open pancreatectomy in rats. Adjustments have been progressively adopted for technical refinement. RESULTS: In five animals a low-cost rat laparoscopic set was used. In other five animals we used a standard laparoscopic set. Three rats died early due to different causes: transection of the gastroesophageal junction, hemorrhagic shock and inadvertent colonic injury. The postoperative period of the other seven rats was uneventful until the seventh postoperative day. DISCUSSION: laparoscopic distal pancreatectomy and splenectomy in rats is feasible and safe, even with a low-cost set, in which the results were alike the standard laparoscopic set.
Assuntos
Laparoscopia , Neoplasias Pancreáticas , Animais , Laparoscopia/efeitos adversos , Laparoscopia/métodos , Modelos Teóricos , Pancreatectomia/efeitos adversos , Pancreatectomia/métodos , Neoplasias Pancreáticas/cirurgia , Ratos , Ratos Wistar , Esplenectomia/efeitos adversos , Esplenectomia/métodosRESUMO
RESUMEN Las metástasis pancreáticas por carcinoma renal de células claras son excepcionales; en general, se presentan como tumores asintomáticos de diagnóstico incidental. Caso clínico: hombre de 61 años, nefrectomizado hace 10 años; durante el seguimiento se detecta una masa pancreática distal de 3 cm, hipervascular. Se realiza pancreatectomía corporocaudal laparoscópica, confirmándose la presencia de una metástasis de carcinoma renal. Las metástasis pancreáticas del carcinoma renal suelen apare cer en forma asintomática y metacrónica. La resección quirúrgica logra buenos resultados oncológicos y debe intentarse siempre que sea posible. En este caso destacamos que se pudo realizar una exéresis radical mediante abordaje mínimamente invasivo.
ABSTRACT Pancreatic metastases from clear cell renal carcinoma are very rare, and generally present as incidental asymptomatic tumors. Case report: a 61-year-old male patient with a history of nephrectomy 10 years before presented a 3-cm hypervascular mass in the distal pancreas during follow-up. A laparoscopic distal pancreatectomy was performed, confirming the presence of metastasis from a renal cell carci noma. Pancreatic metastases from renal cell carcinoma are usually metachronous and asymptomatic. Surgical resection is associated with favorable oncological results and, if possible, should be attempted. In our case, a successful oncological resection could be performed through a minimally invasive ap proach.
Assuntos
Humanos , Masculino , Pessoa de Meia-Idade , Pancreatectomia , Carcinoma de Células Renais/cirurgia , Segunda Neoplasia Primária/cirurgia , Neoplasias Pancreáticas/cirurgia , Esplenectomia/métodos , Espectroscopia de Ressonância Magnética , Tomografia Computadorizada por Raios X , Metástase Neoplásica/diagnósticoRESUMO
Introducción: La esferocitosis hereditaria (ESH) es una anemia hemolítica de observación frecuente, en la cual existen defectos cualitativos o cuantitativos de algunas proteínas de la membrana eritrocitaria que llevan a la formación de hematíes de forma esférica, osmóticamente frágiles, que son atrapados de formas selectiva y destruidos en el bazo, con incidencia variable y más frecuente en pacientes con descendencia europea. Objetivo: Describir la conducta clínica y anestesiológica de un paciente pediátrico con diagnóstico de micro esferocitosis hereditaria programado de forma electiva para procedimiento quirúrgico. Desarrollo: Se presenta un caso clínico de un paciente escolar con diagnóstico de micro esferocitosis hereditaria al cual se le realizó esplenectomía total electiva convencional. Con principal signo dolor a la palpación en hipocondrio izquierdo. Se condujo con anestesia total intravenosa con buenos resultados clínicos quirúrgicos, utilizando propofol a razón de 3 mcg/mL y ketamina a 0,2 mg/mL. La estrategia estuvo basada en cinco aspectos claves: evitar la hipoxia, la hipotermia, la acidosis, reducir la pérdida de sangre, así como un correcto control del dolor postoperatorio. Asociado a lo anterior es indispensable una estrecha vigilancia ya que estos pacientes pueden manifestar crisis hemolítica y aplásica. Conclusiones: El manejo perioperatorio del paciente con esferocitosis hereditaria depende de la severidad del cuadro clínico, de la anemia y su repercusión y del grado de hemólisis. La anestesia total intravenosa es una técnica segura para el tratamiento de pacientes con esferocitosis hereditaria(AU)
Introduction: Hereditary spherocytosis (HS) is a hemolytic anemia of frequent occurrence, in which there are qualitative or quantitative defects of some erythrocyte membrane proteins that lead to the formation of sphere-shaped red blood cells, which are osmotically fragile, and that are selectively trapped and destroyed in the spleen, with variable and more frequent incidence in patients with European descent. Objective: To describe the clinical and anesthesiological behavior of a pediatric patient with a diagnosis of hereditary microspherocytosis electively programmed for a surgical procedure. Development: A clinical case of a school-age patient with a diagnosis of hereditary microspherocytosis was presented. The patient underwent conventional elective total splenectomy. Pain was as the main sign on palpation to the left hypochondrium. The case was conducted with total intravenous anesthesia, with good surgical clinical results, using propofol at a rate of 3 mcg/mL and ketamine at 0.2 mg/mL. The strategy was based on five key aspects: avoid hypoxia, hypothermia, acidosis, reduce blood loss, as well as proper control of postoperative pain. Associated with the above-mentioned, close monitoring is essential, as these patients may manifest hemolytic and aplastic crisis. Conclusions: The perioperative management of the patient with hereditary spherocytosis depends on the severity of the clinical status, the anemia and its repercussion, and the degree of hemolysis. Total intravenous anesthesia is a safe technique for the treatment of patients with hereditary spherocytosis(AU)