Erdheim-Chester disease with chorioretinal and orbital involvement: a case report / Doença de Erdheim-Chester com envolvimento coriorretiniano e orbitário: relato de caso

ABSTRACT A 42-year-old woman presented with bilateral proptosis, chemosis, leg pain, and vision loss. Orbital, chorioretinal, and multi-organ involvement of Erdheim-Chester disease, a rare non-Langerhans histiocytosis, with a negative BRAF mutation was diagnosed based on clinical, radiological, and pathological findings. Interferon-alpha-2a (IFNα-2a) was started, and her clinical condition improved. However, 4 months later, she had vision loss with a history of IFNα-2a cessation. The same therapy was administered, and her clinical condition improved. The Erdheim-Chester disease is a rare chronic histiocytic proliferative disease that requires a multidisciplinary approach and can be fatal if left untreated because of multisystemic involvements.

RESUMO Uma mulher de 42 anos apresentou proptose bi-lateral, quemose, dor nas pernas e perda de visão. Com base em achados clínicos, radiológicos e patológicos, foi diag-nosticada doença de Erdheim-Chester com acometimento orbitário, coriorretiniano e multiorgânico. Trata-se de uma rara histiocitose não Langerhans negativa para a mutação BRAF. Foi iniciado tratamento com interferon alfa-2a (IFNα-2a) e o quadro clínico melhorou. No entanto, quatro meses depois, a paciente apresentou perda visual após a cessação do IFNα-2a. A mesma terapia foi administrada novamente e sua condição clínica melhorou novamente. A doença de Erdheim-Chester é uma doença proliferativa histiocítica crônica rara que necessita de uma abordagem multidisciplinar e pode ser fatal se não tratada, devido a envolvimentos multissistêmicos.

Manejo de neoplasias oncohematológicas en el contexto de la pandemia de COVID-19: recomendación de expertos / Management of oncohematological neoplasms in the COVID-19 pandemic: experts recommendation

RESUMEN La pandemia por COVID-19 originado por el Coronavirus 2 causante de síndrome respiratorio agudo severo (SARS-CoV-2) es causante de una crisis de salud pública a nivel global. Muchos reportes indican resultados desalentadores en pacientes con cáncer respecto a la población general. Por ello, los expertos en el manejo de neoplasias oncohematológicas del Instituto Nacional de Enfermedades Neoplásicas, hospitales nacionales y una clínica privada de Lima Metropolitana han desarrollado recomendaciones obtenidas por consenso para continuar con el manejo de pacientes con neoplasias oncohematológicas en forma segura ante la coyuntura de pandemia.

ABSTRACT The ongoing COVID-19 pandemic caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) is a global public health crisis. Many reports indicate disappointing results in cancer patients compared to the general population. Therefore, experts in the management of oncohematological malignancies from the National Institute of Neoplastic Diseases, national hospitals and a private clinic in Metropolitan Lima have developed recommendations obtained by consensus to continue with the management of patients with oncohematological neoplasms safely in the face of the pandemic.

Liver and Biliary Tract Involvement in Systemic Diseases / 胃肠病学

Liver is vital to human health as it plays a variety of important roles in maintaining homeostasis, including synthesis of essential proteins, generation of bile and bile carrier, regulation of nutrients, as well as the metabolism and excretion functions. Understanding the manifestations of liver and biliary tract involvement in systemic diseases is of great significance not only for the diagnosis and differentiation of systemic diseases, but also for the diagnosis and treatment of the structural and functional changes of the hepatobiliary system. This article reviewed the clinical manifestations of liver and biliary tract involvement in autoimmune diseases, neoplastic diseases, hematological diseases, endocrine system diseases, nervous system diseases, sepsis, cardiovascular diseases, amyloidosis, and granulomatous diseases.

Repercussões da dor social, gênero e pessoas com doença falciforme: um estudo exploratório / Social pain, gender, and people with sickle cell disease: an exploratory study / Dolor social, género y personas con enfermedad falciforme: estudio exploratorio

OBJETIVO: compreender as repercussões da dor social sobre os papéis de gênero de mulheres e homens com doença falciforme. MÉTODO: estudo qualitativo, exploratório, realizado num Centro de Referência em doença falciforme de um município do interior da Bahia. Os dados obtidos de desenhos estória-tema e entrevistas com 16 pessoas foram submetidos à análise de conteúdo temática. RESULTADOS: emergiram duas categorias: "A dor social nas mulheres com doença falciforme se expressa na impossibilidade de ser dona de casa, mãe e mulher sexuada" e "A dor social de homens com doença falciforme se expressa na dificuldade de manter-se trabalhador, ser provedor e homem sexualmente ativo". DISCUSSÃO: a exclusão do trabalho, as alterações da sexualidade e parentalidade interferem nos projetos de vida e mantém a reprodução de estereótipos de gênero. CONCLUSÃO: a escuta sensível numa abordagem de cuidado multiprofissional deve fazer parte do plano terapêutico de pessoas com doença falciforme.

AIM: Social pain, gender, and people with sickle cell disease: an exploratory study. METHOD: this is a qualitative and exploratory study and it was conducted in a Sickle Cell Disease Reference Center in a municipality in the interior of Bahia. The data obtained from theme-story drawings and interviews with 16 people were submitted to thematic content analysis. RESULTS: two categories have emerged: "Social pain in women with sickle cell disease is expressed in the impossibility of being a housewife, mother and woman sexually" and "The social pain of men with sickle cell disease is expressed in the difficulty of being a worker, a provider and a sexually active man". DISCUSSION: the exclusion of work, changes in sexuality and parenting interfere in life projects and maintain the reproduction of gender stereotypes. CONCLUSION: sensitive listening in a multiprofessional care approach should be part of the therapeutic plan for people with sickle cell disease.

OBJETIVO: compreender las repercusiones del dolor social sobre los papeles de género de mujeres y hombres con enfermedad de las células falciforme. MÉTODO: estudio cualitativo, exploratorio, realizado en un Centro de Referencia en enfermedad falciforme de un municipio del interior de Bahía. Los datos obtenidos de los dibujos historietas-tema y entrevistas con 16 personas se sometieron al análisis de contenido temático. RESULTADOS: emergieron dos categorías: "El dolor social en las mujeres con enfermedad falciforme se expresa en la imposibilidad de ser ama de casa, madre y mujer sexuada" y "El dolor social de hombres con enfermedad falciforme se expresa en la dificultad de mantenerse trabajando, ser proveedor y hombre sexualmente activo". DISCUSIÓN: la exclusión del trabajo, las alteraciones de la sexualidad y la vida parental, interfieren en los proyectos de vida y mantienen la reproducción de estereotipos de género. CONCLUSIÓN: la escucha sensibilizada en el abordaje de cuidado multiprofesional debe hacer parte del plan terapéutico de personas con enfermedad de células falciforme.

Difficult splenectomy in 126 cases / 中华普通外科杂志

Objective To evaluate the operative safety of difficult splenectomy.Methods The clinical data of 126 splenectomy cases from January 2005 to December 2011 were analyzed retrospectively.Results All patients were cured and discharged.There was no mortality and pancreatic leakage,no gastric and colonic injury.The postoperative complications occurred in 14 patients including intra-abdominal hemorrhage in 5 cases,pulmonary complications in 7 cases (left pleural effusion and left subphrenic abscess),splenic venous thrombophlebitis in 11 cases and massive ascites in 10 patients.After splenectomy splenic and portal vein thrombi rate in HBV-related cirrhosis was 7％ (6/84) and that was 50％ (6/12) in schistosoma-related cirrhosis (t =13.4,P ＜ 0.01).Conclusions Careful evaluation before operation,skillful technique during operation are determinants for the safety of complex splenectomy.

A case of lymphomatoid granulomatosis with fever, pulmonary nodules, joint pain and superficial lymphadenopathy / 中华老年医学杂志

A 69-year-old man with fever,pulmonary nodules,joint pain and superficial lymphadenopathy was admitted to our hospital.The patient had a history of ten year hypertension.She smoked a pack of cigarettes daily for forty years and quitted for fifteen years.Family history of coronary heart disease,diabetes,cancer or other diseases was negative.Chest CT showed a nodule in the left lung lower lobe.Percutaneous lung biopsy revealed a large number of atypical B cell proliferation and infiltration which involved the vessel wall.The atypical B-cell phenotype and genotype was EBERs (+),CD20 (+),CD30 (+),CD15 (-).The patient was diagnosed as pulmonary lymphomatoid granulomatosis (LYG),an angiodestructive and angioinvasive lymphoproliferative disorder which is an Epstein-Barr virus associated B cell disorder with reactive T lymphocytes.The patient received six courses of chemotherapy.In this rare case,misdiagnosis of LYG often occurred due to the complex clinical presentation and non-specific imaging.Percutaneous or open lung biopsy is the main choice in the diagnosis of LYG.