Pérdida de agudeza visual progresiva en paciente con proptosis. Caso clínico y revisión de la literatura / Progressive visual impairment in a patient with proptosis. Case report and review of the literature.

Introducción: a raíz del siguiente reporte de caso clínico se pretende repensar el diagnóstico diferencial de los tumores orbitales y revisar la literatura existente al respecto. Caso: paciente de 54 años, fumadora, acude a nuestro centro por una pérdida de agudeza visual progresiva de dos años de evolución en el ojo derecho, que se acompañaba de proptosis. Las pruebas de imagen basadas en resonancia magnética y tomografía por emisión de positrones ­ tomografía computarizada (PET-TC) realizadas describían una lesión intraconal derecha de morfología indefinida, que rodeaba el nervio óptico. El estudio inmunohistoquímico y molecular anatomopatológico confirmó la sospecha de síndrome linfoproliferativo extranodal de bajo grado. Discusión: el manejo endoscópico de estas lesiones puede resultar en una menor comorbilidad en comparación con el abordaje externo tradicional. El papel de la cirugía radica en la obtención de una muestra de la lesión que permita un correcto diagnóstico. Conclusiones: el abordaje multidisciplinar con oftalmólogos, hematólogos y expertos en radioterapia permite obtener buenos resultados quirúrgicos y clínicos en la inmensa mayoría de casos.

Introduction: as result of the following clinical case report, we intend to review the differential diagnosis of orbital tumors and review the existing literature in this regard. Case report: a 54-year-old smoking patient, consulted to our department due to a progressive visual impairment over the last two years in her right eye. She presented proptosis in her clinical examination. Imaging studies based on MRI and PET-CT described a right intraconal lesion with an undefined morphology surrounding the optic nerve. Orbital tumors differential diagnosis is delicate. Nevertheless, Non-Hodgkin lymphomas followed by metastasis are the two most common found in this location. The immunohistochemistry and molecular studies, confirmed the suspected diagnosis of extranodal low-grade lymphoproliferative syndrome. Discussion: endoscopic management of these lesions may result in a lower comorbidity compared to traditional external approaches. Role of surgery lays in obtainment of a quality sample which allows a proper diagnosis. Conclusions: multidisciplinary approach with ophthalmologists, hematologists and radiotherapy experts enhance good surgical and clinical results in the vast majority of cases.

Biological therapy and development of neoplastic disease in patients with juvenile rheumatic disease: a systematic review / Uso de imunobiológicos e desenvolvimento de doenças neoplásicas em pacientes com doenças reumáticas juvenis: revisão sistemática

Abstract Juvenile rheumatic diseases affect the musculoskeletal system and begin before the age of 18. These conditions have varied, identifiable or unknown etiologies, but those of an autoimmune inflammatory nature have been associated with an increased risk of development of cancer, regardless of treatment. This study aims to assess, through a systematic review of the literature according to Prisma (Preferred Reporting Items for Systematic Reviews and Meta-Analyses) quality criteria, the risk of cancer in patients with juvenile rheumatic disease, and its association with biological agents. The criteria described by the Strengthening the Reporting of Observational Studies in Epidemiology initiative were used in order to assess the methodological quality of those individual items selected in this study. We analyzed nine publications, from a total of 251 papers initially selected. There was an increase in cancer risk in the population with juvenile rheumatic disease versus the general population. Most specified cancers were of a lymphoproliferative nature. Seven studies did not specify the treatment or not defined an association between treatment and cancer risk. Only one study has suggested this association; in it, their authors observed high risk in patients diagnosed in the last 20 years, a period of the advent of new therapies. One study found an increased risk in a population not treated with biological agents, suggesting a disease in its natural course, and not an adverse effect of therapy. Studies have shown an increased risk of malignancy associated with juvenile rheumatic disease, and this may be related to disease activity and not specifically to the treatment with biological agents.

Resumo As doenças reumáticas juvenis afetam o sistema musculoesquelético e se iniciam antes dos 18 anos. Apresentam etiologia variada, identificável ou desconhecida, porém as de natureza inflamatória autoimune têm sido associadas ao maior risco de desenvolvimento de neoplasias, independentemente do tratamento. Este artigo propõe avaliar, por meio de revisão sistemática da literatura de acordo com os critérios de qualidade Prisma (Preferred Reporting Items for Systematic Reviews and Meta- Analyses), o risco de câncer em pacientes com doenças reumáticas juvenis e sua associação com imunobiológicos. Os critérios descritos pela iniciativa Strengthening the Reporting of Observational Studies in Epidemiology foram usados para avaliar a qualidade metodológica individual dos artigos selecionados no presente estudo. Foram analisadas nove publicações, de 251 incialmente selecionadas. Houve aumento no risco de câncer na população com doença reumática juvenil comparada com a população em geral. A maioria dos cânceres especificados foi de natureza linfoproliferativa. Sete estudos não especificaram a terapêutica ou não definiram associação entre ela e o risco de câncer. Apenas um estudo sugeriu essa associação e observou maior risco em pacientes diagnosticados nos últimos 20 anos, período de advento de novas terapias. Um estudo constatou maior risco em uma população não tratada com imunobiológicos, sugeriu tratar-se da evolução natural da doença, e não do efeito adverso da terapêutica. Os estudos demonstram aumento no risco de malignidade associada a doenças reumáticas juvenis que pode estar relacionada à atividade da doença, e não especificamente ao tratamento com imunobiológicos.

An Adult Case of Severe Chronic Active Epstein-Barr Virus Infection with T-Cell Lymphoproliferative Disorder

No abstract available.

Relative adrenal insufficiency in post-transplant lymphoproliferative disorder.

Post-transplant lymphoproliferative disorder is treated with rapid decrement of immunosuppressive therapy. This cannot be achieved with ease in patients on long-term glucocorticoid therapy, as chronically suppressed adrenal glands may not be capable of mounting adequate response to stress. A 52-year-old Caucasian male presented with fever, orthostatic hypotension, lymphadenopathy and hyponatraemia. Serum cortisol levels were within normal levels with a sub optimal response to stimulation by ACTH. Hyponatraemia and orthostasis responded poorly to fluid restriction, saline and salt repletion but corrected after increasing the steroid dose. The normal baseline cortisol levels represented a stimulated adrenal gland, however, the ACTH stimulation had inadequate response. This sub optimal stimulation and a good response to increased steroids suggest the presence of relative or occult adrenal insufficiency. Relative adrenal insufficiency must be considered in patients who have received prolonged glucocorticoid therapy and have symptoms such as hypotension and/or hyponatraemia.

Desórdenes linfoproliferativos postrasplante hepático (DLPT): hallazgos clínicos e imagenológicos / Lymphoproliferative disorders after liver trasplantation: clinical and diagnostic imaging features

Los desórdenes linfoproliferativos postransplante (DLPT) constituyen un grupo de proliferaciones linfoides que se observan en pacientes transplantados cuyos sistemas inmunes están deprimidos. Se asocian a infecciones por el virus de Epstein Barr (VEB), que puede presentarse como una primoinfección o reactivación de su forma latente. Se analiza un caso de DLPT que se revela como masa abdominal con sus características clínicas e imagenológicas encontrando que los estudios de imágenes realizados (ecografía, RM, colangioRM y videorrectosigmoidescopia) y la punción biopsia constituyen una herramienta fundamental para el diagnóstico precoz y correcto de esta patología

Insuficiência renal aguda infiltrativa causada por doenças linfoproliferativas / Infiltrative acute kidney failure by lymphroproliferative diseases

Relatam-se três casos de insuficiência renal aguda causada por infiltraçäo de células tumorais em parênquima renal. Dois pacientes apresentaram linfoma nao-Hodkin e um paciente apresentou leucemia mielóide crônica em crise blástica. O diagnóstico foi realizado pelo aumento do tamanho renal à ultrassonografia abdominal e por biópsia renal percutânea que demonstrou infiltraçäo dos rins pela neoplasia, tendo sido confirmado pela melhora funcional após a instituiçäo de quimioterapia. Esta causa de insuficiência renal aguda deve ser lembrada em doenças linfoproliferativas, pois apresenta evoluçäo renal favorável desde que o diagnóstico e tratamento sejam estabelecidos em tempo hábil.

Pathology of AIDS in children.

AIDS in children is a multisystem disease. The various infections, degenerative, proliferative and vascular lesions can be classified into three categories based on the known, presumed or undetermined pathogenesis. The primary lesions are due to HIV infection. The associated lesions are related to direct or indirect sequelae of HIV infection or its treatment. The third category is of lesions of undetermined pathogenesis. The pediatric pathologist plays an important role in the study and management of AIDS by demonstrating new pathologic lesions, by making the etiologic diagnosis of infection in children with AIDS, and by providing clinicopathologic correlation which leads to better understanding of the disease process and its natural history. Diagnosis of neoplastic disorders is also made by the pathologist. There is a dearth of systematic pathologic study of AIDS in children in developing countries. Although no basic differences between pathologic lesions in pediatric AIDS in Western countries, and in developing countries is expected, such a study would lead to better understanding and better management of the disorder as it affects children from the developing countries.