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- Primary Liver Lymphoma Presenting as Liver NodulesPublication . Silva, JM; Ornelas Saraiva, R; Simões, G; Araújo, C; Calinas, F; Coimbra, JThe liver is usually affected in advanced stages of lympho-proliferative diseases, but primary liver lymphomas (PLLs) are rare. The diagnosis is usually late, especially in patients without identifiable risk factors, with consequent worse prognosis. We report the case of a 59-year-old female with progressive worsening pain in the right hypochondrium and weight loss. She was previously healthy and had a family history of gastrointestinal and gynecologic neoplasms. During the initial investigation, three liver nodules suggestive of liver metastases were found. Nevertheless, no primary neoplasm was identified in a subsequent evaluation with imaging and endoscopic exams. Laboratory exams excluded hepatic infections, metabolic diseases, and acquired immunosuppression. Biopsy of the lesions revealed diffuse large B-cell lymphoma. Lymph node and medullar involvement were excluded, and the diagnosis of PLL was assumed. The patient started chemotherapy with R-CHOP. With this case, we intend to alert for this differential diagnosis of liver nodules, even in the absence of predisposing conditions for lymphoma. We point out the need to define universal diagnosis criteria for this pathology.
- Presence of Multilobular Necrosis on Liver Biopsy Identifies Corticosteroid Responsiveness in Acute Indeterminate HepatitisPublication . Lin, S; Araújo, C; Hall, A; Kumar, R; Phillips, A; Hassan, M; Engelmann, C; Quaglia, A; Jalan, RBackground and aims: Treatment of patients with severe indeterminate hepatitis (IAH) is an unmet need. Corticosteroids are often used in the management of these patients but criteria for the selection of patients for this intervention are arbitrary. The aims of this study were to analyse the clinical and pathological features of patients with IAH to define predictors of corticosteroid responsiveness. Methods: This study included consecutive patients with acute indeterminate hepatitis admitted to a single hospital and underwent a liver biopsy. The clinical manifestation and histopathological features of steroid and non-steroid groups were compared and their relationship with corticosteroids response was evaluated. Results: Forty-eight patients were included, 24 (50%) recovered and the other half underwent liver transplantation or died within 3-months. Of the 48 cases, 24 received corticosteroids (initial dose of 45 ± 12 mg prednisolone). Corticosteroids were initiated 2.7 ± 3.8 days after admission. Liver biopsy was performed 2-days (median, IQR 1-3) after admission. Fifteen (62.5%) patients receiving corticosteroids survived without transplantation compared with 9 (37.5%) that did not receive steroids (P = .149). In those with multilobular necrosis, 50% reduction in the death/transplantation rate was observed after steroid treatment (P = .018). In patients without multilobular necrosis and with or without perivenulitis, corticosteroids did not impact the outcome. Response to corticosteroids was independent of the MELD score. Conclusions: The presence of multilobular necrosis on liver biopsy helps identify a subgroup of IAH cases who may benefit from the administration of corticosteroids.
- The Modern Value of Clinical AutopsiesPublication . Marques Pontinha, C
- Hyperleukocytosis in Solid Tumors: a Rare Paraneoplastic Syndrome Associated with Poor PrognosisPublication . Ferrão, J; Sardinha, M; Dutra, EHematological paraneoplastic syndromes are fairly uncommon. While mild leukocytosis in solid tumors is well reported, white blood cell (WBC) count over 50,000 u/L, described as paraneoplastic leukemoid reaction (PLR), is not. Indeed, when found, it is usually associated with a higher burden of disease, tumor activity and worse clinical outcomes. We report the case of a challenging and burdensome diagnosis of a presumptive hematological paraneoplastic syndrome in a patient with a locally advanced lung cancer admitted in the Internal Medicine ward. After the end of chemotherapy, clinical and laboratory benefit was observed; however, the aggressive course of the disease became clear, with progression and downhill course that was unresponsive to treatment.
- Anatomical Basis, Histological Findings and Hemodinamics in the Modern Perfusion Model for Human CorpsesPublication . Júnior, E; Bettencourt-Pires, M; Alves, S; Casal, D; Pais, D; Goyri O’neill, J; Vassilenko, VsSeveral modern human cadaveric fixation methods are subject to permanent evaluation. Formaldehyde is the oldest and still the most widely used method of embalming. However, the International Agency for Research on Cancer has proven its high carcinogenic potential and its use was banned, with the recommendation of research for better alternatives in the conservation of corpses. The embalming method of excellence, which preserves all features, while keeping the disinfectant properties against cadaveric decomposition was proposed by João Goyri O’Neill. Their method was considered “the most modern and efficient technique in cadaveric preservation”. The aim of this present study was to analyze the quality of this original perfusion technique, at the organic level, based on central and peripheral hemodynamics. The cadaveric material was embalmed through a pulsed arterial perfusion system, connected to an automatic intermittent pump, that permits stability of the microvascular network, as well as the computerized measurement of the main perfusion parameters, such as flow and pressure. This procedure ensures good preservation of color, elasticity, texture, flexibility and fresh appearance, for several years. The morphological characteristics of the organs exhibited astonishing similarity to the living organic tissues, even several years after embalming and high freezing. Microscopic analysis demonstrated preservation of the structure of vessels, such as the aorta. Further studies on the integrity of the endocardial layer of the heart will enable to adapt the intermittent perfusion pump system to best simulate cardiac rhythm and arterial pulse, during cadaveric surgical training.
- Inflammatory Myofibroblastic Tumour of the Common Bile DuctPublication . Almeida e Sousa, M; Carvalho, A; Mega, R; Bilhim, TInflammatory myofibroblastic tumour is a rare entity of indeterminate biological potential with a reduced tendency for recurrence and metastasis. Although it can arise from multiple organs, the bile duct is a very rare site of origin. We report the case of a 75-year-old asymptomatic male with elevated gamma-glutamyl transferase [1575 U/L (12 - 64 U/L)] and alkaline phosphatase [271 U/L (40 - 150 U/L)]. Computed tomography showed a 17 mm hypervascular lesion in the confluence of the right and left hepatic ducts, with bile duct ectasia and right liver lobe atrophy. The patient was initially managed as having a Klatskin tumour and underwent right hepatectomy. Histology showed a spindle cell proliferation with an inflammatory infiltrate of lymphocytes, plasma cells and collagen-rich stroma, consistent with an inflammatory myofibroblastic tumour. He was discharged 30 days after admission, and nine months later remains asymptomatic. His liver function tests have normalized and follow-up tests are unremarkable.
- Ossificação Cutânea Heterotopica em CriançaPublication . Pereira, B; Freitas, I; Afonso, A; Cardoso, JReporta-se o caso de um doente do sexo masculino observado aos 12 meses de idade por lesões eritemato azuladas deprimidas com 2 a 4 mm de diâmetro no tronco e membros com início aos 3 meses e carácter aditivo. Tinha também, na face posterior da perna direita, placa eritematosa de contornos irregulares, na qual se verificou posteriormente uma textura granulosa. Tratava-se de criança prematura, com história de múltiplas complicações neonatais. A biópsia cutânea revelou ossificação da derme reticular e a Tomografia Computorizada da perna direita imagens lineares de densidade elevada compatíveis com ossificação cutânea. A Ossificação Cutânea é um fenómeno raro sendo na maioria dos casos secundário. A Ossificação Primária é ainda mais rara e as entidades principais em que ela se observa são a Heteroplasia Óssea Progressiva (HOP), a Osteodistrofia Hereditária de Albrigth (OHA) e o Osteoma Cutis em placa (OCP). A HOP caracteriza-se por ossificação com início na derme e em idades precoces e com extensão progressiva para a profundidade ocasionando morbilidade significativa. A OHA associa ossificação da derme e hipoderme não progressiva a alterações fenotípicas (baixa estatura, obesidade, braquidactilia) e hormonais (pseudohipoparatiroidismo). O OCP caracteriza-se pela presença de uma ou mais placas de ossificação da derme e hipoderme não progressivas e sem morbilidade significativa. O diagnóstico definitivo deste caso torna-se difícil, neste momento, uma vez que a HOP pode ter evolução lenta ao longo de anos mimetizando em fases iniciais um OCP; apenas o follow-up as poderá diferenciar em definitivo conforme se verifique progressão ou não da ossificação. A hipótese de HOP parece, contudo, mais provável e a confirmar-se, tanto quanto é do conhecimento dos autores, tratar-se-á do primeiro caso reportado em Portugal.
- Doença de Crohn - Ulcerações Genitais MúltiplasPublication . Cunha, D; Rodrigues, A; Afonso, A; Vieira, A; Freitas, J; Cardoso, JAs ulcerações genitais são frequentemente motivo de envio à consulta de Venereologia. A Doença Inflamatória Intestinal, mesmo na ausência de sintomatologia digestiva, deverá ser sempre uma hipótese de diagnóstico diferencial. A Doença de Crohn é uma doença granulomatosa inflamatória do intestino, crónica e idiopática. A sintomatologia é maioritariamente digestiva, no entanto esta patologia pode ser acompanhada ou precedida de manifestações extraintestinais. Identificam-se lesões mucocutâneas em cerca de 10% dos doentes quando do diagnóstico da doença intestinal, as quais podem constituir patologias específicas ou reactivas. Observou-se uma doente de 28 anos de idade, natural de Cabo Verde, por múltiplas ulcerações, na maioria grosseiramente lineares, localizadas ao períneo, região perianal e sagrada. As lesões eram dolorosas, francamente exsudativas e acompanhavam-se de queixas de astenia, anorexia e perda ponderal. Foi efectuada biópsia de uma úlcera e o exame histopatológico da mucosa intestinal revelou um processo inflamatório com múltiplas células gigantes multinucleadas embora sem verdadeiros granulomas. A colonoscopia e o exame histopatológico da biópsia de mucosa intestinal confirmaram o diagnóstico de Doença de Crohn. O estudo laboratorial efectuado permitiu excluir patologias infeciosas como as Doenças de Transmissão Sexual e a Tuberculose. Durante o internamento a doente teve ainda uma dermatose clinicamente sugestiva de Síndroma de Sweet (que se confirmou no exame histopatológico da biópsia de uma das lesões) e uma perturbação psiquiátrica reactiva grave, com ideação suicida.
- Atypical Presentation of Langerhans Cell HistiocytocisPublication . Marujo, F; Oliveira, MI; Martins, M; Brito, MJ
- Infeção Cutânea e Pulmonar por Mycobacterium AfricanumPublication . Francisco, T; Cunha, D; Vieira, R; Afonso, A; Brito, MJA tuberculose cutânea é uma doença incomum, constituindo 1,5% de todas as formas de tuberculose. Na doença pulmonar e cutânea, as infeções causadas por Mycobacterium africanum e Mycobacterium tuberculosis podem ser clinicamente indistinguíveis. Um rapaz de 6 anos da Guiné-Bissau foi hospitalizado devido a uma úlcera pré-auricular direita com evolução de dois anos e linfadenopatia regional. O teste tuberculínico foi positivo e a radiografia de tórax revelou um nódulo hipotransparente no lobo pulmonar direito, com características sólidas na TAC. As biópsias da úlcera e da linfadenopatia revelaram infiltrado linfocítico com reação granulomatosa; o exame cultural e a PCR confirmaram infeção por Mycobacterium africanum. Foi realizado tratamento antibacilar durante 6 meses, com redução significativa da lesão. Nos países desenvolvidos é importante relembrar as várias formas de tuberculose cutânea que se pode apresentar isoladamente ou em associação com outras formas de infeção. Nos doentes provenientes de áreas endémicas, um teste tuberculínico positivo deve levantar a suspeita desta entidade.