Although hemophagocytic syndrome is a rare clinical condition, it is associated with high mortality and the number of cases described in the literature has progressively increased. The diagnosis of hemophagocytic syndrome is made on the basis of a finding of hemophagocytosis. Sarcoidosis is a highly prevalent disease whose course and prognosis might correlate with the initial clinical presentation and the extent of the disease. We report the case of a patient with long-standing sarcoidosis who presented with intermittent fever and fatigue. The diagnosis of hemophagocytic syndrome was made by bone marrow aspiration, and specific treatment was ineffective. This is the third case of sarcoidosis-related hemophagocytic syndrome reported in the literature and the first reported in Latin America. All three cases had unfavorable outcomes.Keywords: Lymphohistiocytosis, hemophagocytic; Ferritins; Sarcoidosis, pulmonary; Macrophage activation syndrome. ResumoEmbora seja uma condição clínica rara, a síndrome hemofagocítica é associada com alta mortalidade e o número de casos descritos na literatura vem aumentando progressivamente. O diagnóstico de síndrome hemofagocítica depende da presença de hemofagocitose. A sarcoidose é uma doença de alta prevalência cujo curso e prognóstico podem correlacionar-se com a apresentação clínica inicial e a extensão da doença. Relatamos o caso de um paciente com sarcoidose de longa duração que apresentava febre intermitente e fadiga. O diagnóstico de síndrome hemofagocítica foi realizado por aspirado de medula óssea, e o tratamento específico foi ineficaz. Trata-se do terceiro caso de síndrome hemofagocítica relacionada a sarcoidose na literatura mundial e o primeiro na literatura latino-americana. Os três casos tiveram desfecho desfavorável. blood workup revealed leukocytosis (neutrophils, 32%). Anemia and thrombocytopenia were found. Electrolytes, creatinine, and urea were normal, and blood and urine cultures were negative (Table 1). The patient was hospitalized for further investigation. He had a medical history of diabetes and dyslipidemia but reported no allergies, blood transfusions, smoking, or alcohol consumption. DescritoresAt admission, the patient was febrile (39°C) and a little anxious. He had no jaundice, rash, or lymphadenopathy. His blood pressure was 130/70 mmHg, his RR was 21 breaths/min, his SaO 2 was 96%, and his HR was 88 bpm. Cardiovascular examination was normal. Pulmonary examination revealed normal breath sounds. Physical examination was otherwise unremarkable. Laboratory test results at admission were similar doses of prednisone (10 mg/day) in order to control the activity of the disease. The patient had been well until March of 2010, when he had episodes of fever. He received emergency room treatment, the amoxicillin-clavulanate combination having been prescribed. The fever did not subside. Ten days later, the patient sought emergency room treatment again (in the same emergency room) and received levofloxacin. The fever persisted, followed by mild dyspnea, a...
Introduction:Diagnostic approaches to patients with a pleural effusion must be precise because many procedures depend on the nature of the fluid in the effusion. To date, no biochemical test is considered an appropriate alternative to Light’s criteria. This study compared the absolute pleural cholesterol (PC) level and the pleural cholesterol/serum cholesterol (PC/SC) ratio with Light’s criteria to determine exudative pleural effusions.Materials and Methodology:This study was a case series of 100 consecutive patients with pleural effusions. The clinical parameters that were used to diagnosis an exudative effusion included the cholesterol level, a pleural cholesterol level ≥ 50 mg/dL, a pleural/serum ratio ≥ 0.4, and Light’s criteria. The sensitivity, specificity, and positive and negative predictive values of each test for the diagnosis of an exudative effusion were assessed.Results:A total of 79 patients were definitively diagnosed with an exudative effusion and were included in the trial and analyzed. The mean PC level in the exudates was 90.39 mg/dL. The PC levels demonstrated a sensitivity of 97.22%, a specificity of 85.71%, a positive predictive value of 98.59% and a negative predictive value of 75%. The PC/SC ratio demonstrated a sensitivity of 81.48%, a specificity of 57.14%, a positive predictive value of 93.61% and a negative predictive value of 28.57%.Conclusion:The pleural cholesterol dosage level and the pleural/serum cholesterol ratio can be utilized as unique biomarkers to identify an exudative effusion and replace Light’s criteria.
O sequestro pulmonar é uma rara anomalia congênita, caracterizada por tecido pulmonar embrionário não funcionante, perfazendo 0,15-6,40% de todas as malformações pulmonares congênitas. Essa anomalia envolve o parênquima e a vascularização pulmonar, sendo classificado como intralobar ou extralobar. Neste relato, descrevemos o caso de um paciente de 56 anos com hemoptise e imagem hipotransparente retrocardíaca em base de hemitórax esquerdo na radiografia de tórax. Após a realização de TC com contraste endovenoso, foi evidenciada a presença de sequestro pulmonar. O paciente foi submetido à cirurgia para a retirada do tecido anômalo, que foi realizada com sucesso. Apresentou boa evolução pós-operatória e recebeu alta com acompanhamento ambulatorial.
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