Absceso esplénico en un paciente adulto. Reporte de caso

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.

Procedimiento anestésico-quirúrgico en un adulto con esplenomegalia por linfoma primario de bazo / Anesthetic-surgical procedure in an adult with splenomegaly due to primary spleenic lymphoma: en

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.

Recent Research Advance to Differentiate Portal Hypertension Associated with Primary Myelofibrosis and Cirrhosis --Review / 中国实验血液学杂志

Primary myelofibrosis (PMF) is easily confused with cirrhosis, due to its main clinical manifestations of splenomegaly and the blood cytopenia. This review focuses on clinical studies to identify primary myelofibrosis and cirrhosis related portal hypertension, to analyze the differences between the two diseases, in order to distinguish PMF and cirrhosis from the pathogenesis, clinical manifestations, laboratory examinations and treatment principles, and simultaneously improve clinicians' understanding of PMF, which is a reference for exploring the early screening or diagnostic indicators of PMF, also provides a clinical basis for the application of new targeted drugs such as ruxolitinib.

A family of Niemann-Pick disease type C caused by compound heterozygous mutations in NPC1 gene / 中华神经科杂志

Objective:To report the clinical and genetic characteristics of a family with Niemann-Pick disease type C caused by novel compound heterozygous mutations in the NPC1 gene to improve the clinicians′ recognition of the disease. Methods:Two patients from the family with non-consanguineous marriages admitted to the Department of Neurology of the First Affiliated Hospital of Kunming Medical University in 2020 were examined in detail. Peripheral blood DNA was extracted, and whole exome sequencing was performed on the patients, combined with Sanger sequencing for verification. The mutation and protein function predictor softwares were applied to analyze the mutation sites.Results:The inheritance was autosomal recessive in this family. The onset age of the proband was 9 years, and the main clinical manifestations were dysarthria, dysphagia, cognitive impairment, ataxia, bilateral pyramidal tract impairment, vertical supranuclear gaze palsy and splenomegaly. The clinical phenotype of the proband′s younger brother was similar to that of the proband, but it was more severe than that of the proband. The younger brother of the proband had an earlier age of onset and severe psychomotor retardation. Whole exome sequencing showed that both brothers carried 2 rare variants of NPC1 gene:1 pathogenic, stop gain at c.352_353del, p.Gln119ValfsTer8, and a missense change, c.593A>G, p.Asn198Ser, of suspected pathogenic. Sanger sequencing confirmed that compound heterozygous mutations were derived from the proband′s parents. According to the American College of Medical Genetics and Genomics guidelines, the above variants were rated as pathogenic and suspected pathogenic, respectively. And the c.593A>G, p.Asn198Ser mutation found in this family was a novel one which had not been reported yet. The proband had delayed diagnosis for 7 years from the onset of symptoms. After taking megastat for 1 year, the symptoms of dysphagia, ataxia and vertical eye movement disorder were significantly improved. Conclusions:The clinical phenotype of the pedigree was consistent with the clinical phenotype of Niemann-Pick disease type C. Compound heterozygous mutations of NPC1 gene (c.352_353del; c.593A>G) were found to be the genetic cause of the family.

A case report of cirrhosis complicated with spontaneous spinal cord hemorrhage and literature review / 中华神经科杂志

A case of cirrhosis with spontaneous spinal cord hemorrhage is reported. The patient was a 57-year-old male, whose main clinical symptoms were acute onset, sudden progressive decline of lower limb muscle strength, and incontinence. Magnetic resonance imaging+magnetic resonance angiography+susceptibility-weighted imaging of thoracic spinal cord showed that thin strip like equal and slightly high signal could be seen in the spinal cord of T 1WI sequence, a few low signal foci could be seen in the corresponding part of T 2WI sequence, and there was no obvious change in enhanced scanning. Obvious long strip like low signal foci were shown in the spinal cord of susceptibility-weighted imaging, and the range was significantly larger than that of T 2WI sequence. Spinal cord hemorrhage was diagnosed before operation, and no obvious improvement was found after drug treatment. Spontaneous spinal cord hemorrhage was diagnosed after surgical incision.

The muddled pancytopenia

Pancytopenia always remains a challenge for its detailed diagnostics evaluation, ranging from megaloblastic anemia to marrow aplasia and leukemia. Here, we report the case scenario of a 70-year-old male who presented with pancytopenia and his elaborated diagnostics approach, concluding the diagnosis of myelofibrosis with positive W515L. This case illustrates the uniqueness of establishing the diagnosis of myelofibrosis.

The left gastro-omental vessels are able to maintain the entire spleen blood supply / Os vasos gastromentais esquerdos são capazes de manter o suprimento sanguíneo de todo o baço

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.

Multidisciplinary Treatment on a Case of ROSAH Syndrome / 罕见病研究

A 15-year-old female was referred to the hospital with intermittent fever, where multiple systemic abnormalities were found, such as splenomegaly, secondary hypersplenism, retinitis pigmentosa, and ectodermal dysplasia. Medical history revealed that she had suffered recurrent respiratory infections, blurred vision at night, and dysplasia of teeth and nail beds since childhood. Then she was suspected to be experiencing ROSAH syndrome, a rare disease newly recognized in recent years, which was finally confirmed by gene sequencing results. During a course of treatment with tumor necrosis factor inhibitors, recurrent fever with elevated inflammatory markers reappeared, and the child developed headaches. To guide the comprehensive treatment and improve the patient's quality of life, the multidisciplinary team in Peking Union Medical College Hospital discussed together and directed the following treatment.

Esplenomegalia gigante por enfermedad de Gaucher

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.

Angiossarcoma hepático primário: uma trágica evolução ­ relato de caso e revisão de literatura / Primary hepatic angiosarcoma: a tragic evolution ­ case report and literature review / Angiosarcoma hepático primario: una evolución trágica ­ reporte de caso y revisión de la literatura

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.

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

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

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

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

The spleen size in patients undergoing hemodialysis / O tamanho do baço em pacientes em hemodiálise

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.

Síndrome de Kasabach-Merritt esplénico en adulto. Presentación de un caso y revisión del tema / Splenic Kasabach-Merritt syndrome in an adult. Presentation of a case and review of the topic / Síndrome de Kasabach-Merritt esplênica em adulto. Apresentação de um caso e revisão do tema

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.

Clinical Analysis of Oxaliplatin-related Thrombocytopenia in Patients with Digestive System Malignancy / 肿瘤防治研究

Objective To investigate the changing trend and correlation of platelet count and spleen diameter in patients with digestive system malignancy receiving oxaliplatin-based chemotherapy. Methods We retrospectively analyzed clinical data of 72 patients with digestive system cancer, recorded and analyzed platelet count and spleen diameter during and after oxaliplatin-based chemotherapy. Results The incidence of thrombocytopenia in all patients was 65.3%. The median time of thrombocytopenia after the beginning of chemotherapy was 2.53±0.49 months, and the median cumulative dose of oxaliplatin was 520±35.81 mg/m2; the median time of lowest platelet count after the beginning of chemotherapy was 4.03±0.49 months, and the median cumulative dose of oxaliplatin was 780±36.32 mg/m2. Splenomegaly occurred in 52(72.2%) patients during the follow-up. The median increase rate was (18.82±0.01)%. The median time of splenomegaly after the beginning of chemotherapy was 2.15±0.19 months, the median time for the largest spleen diameter was 4.68±2.89 months; after the end of chemotherapy, the median time for spleen contraction was 3.28±0.44 months, and the median time for spleen recovery was 8.80±1.05 months. Conclusion Oxaliplatin-based chemotherapy can cause thrombocytopenia and splenomegaly, and it is difficult to recover to baseline for a long time after the end of chemotherapy. The increase of spleen diameter was positively correlated with splenomegaly and thrombocytosis.

Linfoma hepatoesplénico de células T en un paciente adolescente / Hepatosplenic T-cell lymphoma in an adolescent patient

Resumen Introducción: Los linfomas de células T son infrecuentes y se caracterizan por presentarse en la población de los adultos jóvenes. Además, suele acompañarse de patologías como la anemia moderada, hepatoesplenomegalia y trombocitopenia e infiltración sinusoidal por linfocitos T en células de médula ósea, bazo e hígado. Caso clínico: Se presenta un caso clínico de un adolescente que tiene los síntomas característicos de esta patología, con sospecha clínica y diagnóstico paraclínico confirmado con histoquímica de médula ósea. Conclusión: Es una entidad infrecuente de pronóstico desfavorable, hasta el momento el paciente está estable recibiendo tratamiento. Para utilizar el enfoque adecuado en el diagnóstico y brindar tratamiento, es necesario considerar todos los hallazgos clínicos.

Abstract Introduction: T-cell lymphomas are uncommon; these tend to be present in young adult patients. Additionally, this condition is characterized by the existence of pathologies like moderate anemia, hepatosplenomegaly disorder, thrombocytopenia and sinusoidal infiltration by T-Lymphocytes in bone marrow cells, spleen and liver. In this study a case of this rare lymphoma is going to be presented. Case report: A clinical case of an adolescent who presents the characteristic symptoms of this pathology is exposed. This clinical suspicion held a paraclinical diagnosis that was confirmed by histochemistry of bone marrow tests. Conclusion: It is an infrequent condition with an unfavorable prognosis. Until now the patient remains stable and is receiving treatment, the clinical findings of the disease raise awareness about the importance of carrying out the appropriate diagnosis procedures and providing treatment.

Características clínicas, epidemiológicas, diagnóstico y tratamiento de los pacientes pediátricos con histoplasmosis en el Hospital Materno infantil José Domingo de Obaldía, desde enero 2008 a diciembre 2018 / Clinical, epidemiological characteristics, diagnosis and treatment of pediatric patients with histoplasmosis at the Hospital Materno Infantil José Domingo de Obaldia, since January 2008 to December 2018

Introducción: La histoplasmosis es una enfermedad causada por el hongo Histoplasma capsulatum descrita por primera vez por Samuel Taylor Darling en un trabajador de la zona del canal de Panamá. La exposición al hongo es frecuente en áreas tropicales y subtropicales, la mayoría son infecciones asintomáticas o ligeramente sintomáticas Los pacientes pediátricos son más vulnerables a padecer formas graves de histoplasmosis, especialmente menores de dos años. Materiales y métodos: Se realizó un estudio transversal retrospectivo, en pacientes diagnosticados con histoplasmosis en el Hospital Materno Infantil José Domingo de Obaldía de enero de 2008 a diciembre 2018. Resultados:Se analizaron 8 casos en 11 años (2008-2018), El principal grupo afectado fueron los menores de 2 años (n=7). Las características clínicas encontradas fueron: hepatomegalia y esplenomegalia en todos los pacientes, además, fiebre, adenopatías, anorexia, y astenia Se encontró trombocitopenia en todos los casos, seguido de anemia y leucopenia; e infiltrado alveolo intersticial en la radiografía de tórax de 6 pacientes. No se encontraron pacientes con infección por VIH. El método diagnóstico fue a través de estudios histopatológicos en el aspirado de médula ósea. El tratamiento utilizado fue anfotericina B, seguido de Itraconazol. De los 8 pacientes, 6 fallecieron con una letalidad del 75%. Conclusiones: Es una entidad poco frecuente en nuestra institución. Tiene una alta letalidad, siendo los más vulnerables los menores de 2 años; representando el 89% de las defunciones. Los pacientes presentaron datos de infección diseminada. Se debe sospechar histoplasmosis en pacientes con hepatomegalia, esplenomegalia, trombocitopenia, anemia y/o leucopenia.

Introduction: Histoplasmosis is a disease caused by the Histoplasma capsulatum fungus, first described by Samuel Taylor Darling in a worker in the Panama Canal area. Exposure to the fungus is common in tropical and subtropical areas, most are asymptomatic or slightly symptomatic (self-limited) infections. Pediatric patients are more vulnerable to severe forms of histoplasmosis, especially under two years. Materials and methods: A retrospective cross-sectional study was carried out in patients diagnosed with histoplasmosis at Hospital Materno Infantil José Domingo de Obaldía from January 2008 to December 2018. Results: 8 cases were analyzed in 11 years (2008-2018), mostly of the male sex (n = 5). The main age group affected were those under 2 years of age (n = 7). The clinical features found were hepatomegaly and splenomegaly in all patients, in addition, fever, lymphadenopathy, anorexia, and asthenia. In the complementary studies thrombocytopenia was found in all cases, followed by anemia and leukopenia, in addition to alveolo-interstitial infiltrate on the chest radiograph of 6 patients. The diagnostic method was histopathological studies in bone marrow aspirate. The treatment used was amphotericin B, followed by Itraconazole. Of the 8 patients, 6 died with a lethality of 75%. Conclusions: It is a rare entity in our institution. It has a high lethality, the most vulnerable being those under 2 years. Patients presented disseminated infection data. Histoplasmosis should be suspected in patients with hepatomegaly, splenomegaly, thrombocytopenia, anemia and (or ) leukopenia.

Clinico-pathological analysis of lymphoproliferative disorders: a 3 year study

Background: The incidence of lymphoproliferative disorders has increased in many parts of the world. Newer subtypes have been identified by the new WHO classification. Accurate subtyping of lymphomas is crucial for prompt treatment. Objective of the study was to assess the clinicopathological pattern of lymphoproliferative diseases diagnosed in Rajagiri hospital over a period of 3 years.Methods: A retrospective study on all patients who were diagnosed with lymphoma in Rajagiri hospital during January 2016 to December 2018 was conducted and the data were reviewed and analyzed.Results: A total of 151 patients were included in the study. Majority of the subjects (63%) were males. The predominant age group affected was 61-80 years. Mean age group was 58.46 years (SD=19.05 years). Most common presenting symptom was painless lymphadenopathy. B symptoms were seen in 18% of subjects and was found to be more commonly associated with B cell Non-Hodgkin lymphoma. Most common lymph node involved was cervical lymph node, while the most common extra nodal site was bone marrow. Most common lymphoproliferative disorder was Diffuse large B cell Lymphoma. Lymph node involvement was found in 74% of patients, while hepatomegaly and splenomegaly were seen in 11% and 18.5% of patients. 20% of patients had secondary bone marrow involvement. 8 patients developed recurrent lesions involving other organs.Conclusions: Clinicopathologic patterns of lymphoproliferative disorders vary across various regions. A proper understanding of demographical distribution of lymphomas is very essential, as it can provide valuable clues for accurate diagnosis and treatment.

Study on ultrasound observations in children with typhoid fever in a tertiary care hospital

Background: Enteric fever continues to be endemic in poor countries globally, although it has been eradicated from the developed nations due to their well-organized sanitation and protected water supply. The five Fs most concerned with spread of typhoid disease are fingers, food, fomites, flies, and feces. Enteric fever is predominantly caused by Salmonella typhi and next in frequency is Salmonella paratyphi. Very less literature is available on radiological manifestations of typhoid fever in children on the basis of age difference in India. Hence, a study was conducted to observe the radiological findings and to correlate with laboratory manifestations in typhoid fever. Objectives of the study was to observe the ultrasound abdomen changes in Typhoid fever at rural area.Methods: The current study was conducted at Department of pediatrics of Narayana Medical College Hospital, Nellore, Andhra Pradesh state in a period of one year. All patients presenting with fever having positive for Widal test were included. In total 50 patients were included and divided into 2 age groups, <5 years and >5years. The laboratory results and abdominal ultrasound were conducted in the two groups and compared.Results: Total 20 patients in <5 years age and 30 patients in >5 years were enrolled. Male to female ratio was 2:1 in both groups. Common laboratory findings showed 32 patients (64%) with Anemia, elevated liver enzymes in 40 patients (80%), and elevated ESR in 42 patients (84%). 45 patients have Splenomegaly and hepatomegaly with normal parenchymal echotexture. Gallbladder sludge and biliary sludge was seen in 6 patients. Thickened gall bladder in 34 patients (68%) was observed. Bowel wall thickening seen in 32 patients (64%). mesenteric lymphadenopathy in 36 patients (68%) observed. All were recovered by treatment.Conclusions: On ultrasound, splenomegaly, hepatomegaly, and thick-walled gallbladder were observed in most of the childrens with typhoid fever. Therefore, ultrasound can also be used as supportive diagnose along with laboratory parameters due to it is a non-invasive and economical tool of diagnosing typhoid.

Study of leukocyte profile in patients of fever with splenomegaly

Background: There are many different possible etiologies of fever with splenomegaly; few patients with prolonged fever will remain undiagnosed despite an intensive diagnostic work-up. The presence of splenomegaly in patients with fever narrows down the differential diagnosis. Objectives: The objectives of this study were as follows: (I) To evaluate the causes of fever with splenomegaly and (II) to study the leukocyte profile which includes pattern of total leukocyte count and differential leukocyte count (DLC) in various diseases presenting as fever with splenomegaly. Materials and Methods: A total of 100 patients with fever and splenomegaly were included in the study. Complete hematological study such as complete blood count provided with main focus on total leukocyte count and DLC including neutrophil, basophil, eosinophil, lymphocyte, and monocyte and peripheral blood smear to especially note the morphology of white blood cells (WBCs) and to determine their relative percentage in blood. Results: A total of 100 patients of fever with splenomegaly with male and female ratio of 1.7:1 were included in the study. While considering age-wise distribution, the highest percentage of cases was fell in the age group of 21–30 years. Infectious causes constituted 55% of cases, followed by hematological malignancies constituting 33% of cases. Other causes included 12% of cases. Kala-azar was the most common cause of fever with splenomegaly in our study followed by malaria and acute myeloid leukemia. Pancytopenia was found in 29% of cases and followed by 38% of bicytopenia cases in our study. About 32% of cases presented with normal total leukocyte count, 39% of cases showed leukopenia, and 29% of cases showed leukocytosis. Leukopenia was commonly found in kala-azar followed by hypersplenism. Neutropenia was most commonly present in kala-azar. Conclusion: This study helps in understanding the distribution of leukocyte count and DLC pattern in various diseases presented as fever with splenomegaly and also the variable presenting signs and symptoms of these diseases so that the patients of fever with splenomegaly investigated and treated in a proper manner.

Infarto esplénico masivo en un paciente pediátrico sin factores predisponentes / Massive splenic infarct in a pediatric patient without predisposing factors

El infarto esplénico es una entidad rara en la edad pediátrica que, cuando se encuentra, se asocia a otros factores predisponentes, como enfermedades oncológicas, hematológicas o infecciosas. Su presentación es asintomática o sintomática con dolor abdominal, fiebre y esplenomegalia. No existe consenso sobre cuál es la mejor opción de tratamiento, y se decide, en la mayoría de los casos, un manejo conservador o quirúrgico de acuerdo con las características individuales del paciente. Se presenta el caso de un paciente escolar que acude por un cuadro de dolor abdominal agudo y fiebre con diagnóstico de infarto esplénico masivo sin etiología aparente que fue manejado de manera conservadora, con evolución favorable. Es importante considerar este padecimiento como causa de dolor abdominal asociado a esplenomegalia y destacar la relevancia de esta presentación de caso, ya que se presenta sin relación con enfermedades subyacentes.

Splenic infarction is a rare entity in the paediatric age that, when found, is associated with other predisposing factors such as oncological, haematological or infectious diseases. It is whether asymptomatic or symptomatic with abdominal pain, fever and splenomegaly. There is no consensus on which is the best treatment option, in most cases deciding conservative or surgical management according to individual patient characteristics. We present the case of a patient with acute abdominal pain and fever with a diagnosis of massive splenic infarction without apparent aetiology that was managed conservatively with favourable evolution. It is important to consider this condition as a cause of abdominal pain associated with splenomegaly.