Acute erythroid leukemia: autopsy report of a rare disease

Acute erythroid leukemia (AEL) is a rare subtype of acute myeloid leukemia(AML), characterized by predominant erythroid proliferation. The 2008 WorldHealth Organization (WHO) classification of AML defined two AEL subtypes:erythroleukaemia (EL), in which erythroid precursors account for 50% or moreof all nucleated bone marrow cells and myeloblasts account for 20% or more ofthe nonerythroid cell population; and pure erythroid leukemia (PEL), in whicherythroid precursors account for 80% or more of all nucleated bone marrowcells. We report the case of an elderly female patient with wasting syndromeand pancytopenia without evidence of blasts in peripheral blood. A diagnosisof PEL was established on the basis of bone marrow biopsy findings. Thepatient died on postadmission day 20, and an autopsy was performed. Wereclassified the disease as EL on the basis of the autopsy findings, whichincluded myeloblasts accounting for more than 20% of the nonerythroid cellsin the bone marrow, as well as leukemic infiltration and myeloid metaplasia insolid organs, such as the liver, spleen, kidneys, adrenal glands, and abdominallymph nodes. A rare disease, AEL accounts for less than 5% of all AMLs and ispractically a diagnosis of exclusion. Autopsy reports of AEL are extremely rarein the literature. We demonstrate that in the case reported here, leukemia cellstended to infiltrate solid organs with myeloid metaplasia. Our findings alsoshow that a larger neoplastic bone marrow sample is crucial to the correctdiagnosis of EL, which is based on morphological and quantitative criteria

Hemophagocytic lymphohistiocytosis of indeterminate cause: a fatal adult case

Hemophagocytic lymphohistiocytosis (HLH) is an uncommon life-threatening disorder characterized by wide spread non-neoplastic proliferation and inappropriate activation of mature macrophages resulting in hypercytokinemia.This uncontrollable and ineffective systemic immune response causes fever, hepatosplenomegaly, cytopenias and subsequently multiorgan failure. The authors report a case of a 41-year-old male patient with a 30-day history of weight loss, fever, icterus, hepatomegaly, and cytopenias. The diagnosticworkup disclosed hypertriglyceridemia, hypofibrinogenemia, and elevated ferritin. Bone marrow examination and clinical course raised the suspicion of HLH and treatment was started with high-dose corticosteroids and immune globulin. The patient underwent multi-organ failure and expired after 58 days ofhospitalization. The autopsy finding included massive bone marrow infiltration by non-neoplastic histiocytes, many of them showing hemophagocytosis, which immunohistochemical study revealed diffuse CD68-positive histiocytes, which were negative for S100 protein. Hemophagocytosis was also observedin the lungs, lymph nodes and liver. The immediate cause of death was attributed to a massive intestinal bleeding due to extensive ischemic necrosis at the duodenum/jejunal transition area.nul

Incidence, predictors, risk scores of cardiovascular complications, and total death rate within 30 days and 1 year after elective arterial surgery

Introdução: Estima-se que ocorram 2,5 milhões de mortes por ano relacionadas a cirurgias não cardíacas e cinco vezes este valor para morbidade, com limitações funcionais e redução na sobrevida em longo prazo. Pacientes que deverão ser submetidos à cirurgia vascular são considerados de risco aumentado para eventos adversos cardiovasculares no pós-operatório. Há, ainda, muitas dúvidas em como fazer uma avaliação pré-operatória mais acurada desses pacientes. Objetivo: Em pacientes submetidos à cirurgia vascular arterial eletiva, avaliar a incidência e preditores de complicações cardiovasculares e/ou óbito total, e calcular a performance dos modelos de estratificação de risco mais utilizados. Métodos: Em pacientes adultos, consecutivos, operados em hospital terciário, determinou-se a incidência de complicações cardiovasculares e óbitos, em 30 dias e em um ano. Comparações univariadas e regressão logística avaliaram os fatores de risco associados com os desfechos e a curva ROC (receiver operating characteristic) examinou a capacidade discriminatória do Índice de Risco Cardíaco Revisado (RCRI) e do Índice de Risco Cardíaco do Grupo de Cirurgia Vascular da New England (VSG-CRI). Resultados: Um total de 141 pacientes (idade média 66 anos, 65% homens) realizou cirurgia de: carótida 15 (10,6%), membros inferiores 65 (46,1%), aorta abdominal 56 (39,7%) e outras (3,5%). Complicações cardiovasculares e óbito ocorreram, respectivamente, em 28 (19,9%) e em 20 (14,2%), em até 30 dias, e em 20 (16,8%) e 10 (8,4%), de 30 dias a um ano. Complicações combinadas ocorreram em 39 (27,7%) pacientes em até 30 dias e em 21 (17,6%) de 30 dias a um ano da cirurgia. Para eventos em até 30 dias, os preditores de risco encontrados foram: idade, obesidade, acidente vascular cerebral, capacidade funcional ruim, cintilografia com hipocaptação transitória, cirurgia aberta, cirurgia de aorta e troponina alterada. Os escores Índice de Risco Cardíaco Revisado (RCRI) e Índice de Risco Cardíaco do Grupo de Estudo Vascular da New England (VSG-CRI) obtiveram AUC (area under curve) de 0,635 e 0,639 para complicações cardiovasculares precoces e 0,562 e 0,610 para óbito em 30 dias, respectivamente. Com base nas variáveis preditoras aqui encontradas, testou-se um novo escore pré-operatório que obteve AUC de 0,747, para complicações cardiovasculares precoces, e um escore intraoperatório que apresentou AUC de 0,840, para óbito em até 30 dias. Para eventos tardios (de 30 dias a 1 ano), os preditores encontrados foram: capacidade funcional ruim, pressão arterial sistólica, cintilografia com hipocaptação transitória, ASA (American Society of Anesthesiologists Physical Status) classe > II, RCRI (AUC 0,726) e troponina alterada. Conclusões: Nesse grupo pequeno e selecionado de pacientes de elevada complexidade clínica, submetidos à cirurgia vascular arterial, a incidência de eventos adversos foi elevada. Para complicações em até 30 dias, mostramos que os índices de avaliação de risco mais utilizados até o momento (RCRI e VSG-CRI) não apresentaram boa performance em nossa amostra. A capacidade preditiva de um escore mais amplo pré-operatório, e uma análise de risco em dois tempos: no pré-operatório e no pós-operatório imediato, como o que simulamos, poderá ser mais efetiva em estimar o risco de complicaçõesIntroduction: Approximately 2.5 million deaths are caused by non-cardiac surgeries per year, while morbidity, represented by functional impairment and a decline in long-term survival, accounts for five times this value. Patients who require a vascular surgery are considered at an increased risk for adverse cardiovascular events in the postoperative period. However, the method for obtaining a more accurate preoperative evaluation in these patients has not yet been determined. Objective: In patients undergoing elective arterial vascular surgery, the incidence and predictors of cardiovascular complications and/or total death were determined and the performance of risk stratification models was assessed. Methods: The incidence of cardiovascular complications and death within 30 days and 1 year after vascular surgery was determined in consecutive adult patients operated in a tertiary hospital. Univariate comparison and logistic regression analysis were used to evaluate risk factors associated with the outcome, and the receiver operating characteristic (ROC) curve determined the discriminatory capacity of the Revised Cardiac Risk Index (RCRI) and the Cardiac Risk Index of the New England Vascular Surgery Group (VSG-CRI). Results: In all, 141 patients (mean age, 66 years; 65% men) underwent vascular surgery, namely for the carotid arteries (15 [10.6%]), inferior limbs (65 [46.1%]), abdominal aorta (56 [39.7%]), and others (5 [3.5%]). Cardiovascular complications and death occurred in 28 (19.9%) and 20 (14.2%) patients, respectively, within 30 days after surgery, and in 20 (16.8%) and 10 (8.4%) patients, respectively, between 30 days and 1 year after the surgical procedure. Combined complications occurred in 39 patients (27.7%) within 30 days and in 21 patients (17.6%) between 30 days and 1 year after surgery. The risk predictors for cardiovascular events that occurred within 30 days were age, obesity, stroke, poor functional capacity, transitory myocardial hypocaptation on scintigraphy, open surgery, aortic surgery, and abnormal troponin levels. The RCRI and VSG-CRI showed an under the curve area of 0.635 and 0.639 for early cardiovascular complications as well as of 0.562 and 0.610 for death within 30 days, respectively. Based on the predictors found in this study, a new preoperative score was proposed, based on an AUC of 0.747 obtained for early cardiovascular complications and an intraoperative score that presented an AUC of 0.840 for death within 30 days. For late events (between 30 days and 1 year), the predictors were poor functional capacity, systolic blood pressure, presence of transitory myocardial hypocaptation on scintigraphy, class > II American Society of Anesthesiologists Physical Status score, RCRI (AUC= 0.726), and abnormal troponin levels. Conclusions: In this small group of patients with increased clinical complexity who underwent arterial surgery, the incidence of adverse events was high. In our series, we found that RCRI and VSG-CRI do not reasonably predict the risk of cardiovascular complications. The predictive capacity of a modified preoperative score and evaluating the risk preoperatively and early postoperatively, such as that simulated in this study, may be more effective in determining the risk of complication

Incidence and Predictors of Cardiovascular Complications and Death after Vascular Surgery

AbstractBackground:Patients undergoing arterial vascular surgery are considered at increased risk for post-operative complications.Objective:To assess the incidence and predictors of complications and death, as well as the performance of two models of risk stratification, in vascular surgery.Methods:This study determined the incidence of cardiovascular complications and deaths within 30 days from surgery in adults. Univariate comparison and logistic regression assessed the risk factors associated with the outcomes, and the receiver operating characteristic (ROC) curve assessed the discriminatory capacity of the revised cardiac risk index (RCRI) and vascular study group of New England cardiac risk index (VSG-CRI).Results:141 patients (mean age, 66 years; 65% men) underwent the following surgeries: carotid (15); lower limbs (65); abdominal aorta (56); and others (5). Cardiovascular complications and death occurred within 30 days in 28 (19.9%) and 20 (14.2%) patients, respectively. The risk predictors were: age, obesity, stroke, poor functional capacity, altered scintigraphy, surgery of the aorta, and troponin change. The scores RCRI and VSG-CRI had area under the curve of 0.635 and 0.639 for early cardiovascular complications, and 0.562 and 0.610 for death in 30 days.Conclusion:In this small and selected group of patients undergoing arterial vascular surgery, the incidence of adverse events was elevated. The risk assessment indices RCRI and VSG-CRI did not perform well for complications within 30 days

Pre-operative evaluation of adults undergoing elective noncardiac surgery Updated guideline from the European Society of Anaesthesiology

The purpose of this update of the European Society of Anaesthesiology (ESA) guidelines on the pre-operative evaluation of the adult undergoing noncardiac surgery is to present recommendations based on the available relevant clinical evidence. Well performed randomised studies on the topic are limited and therefore many recommendations rely to a large extent on expert opinion and may need to be adapted specifically to the healthcare systems of individual countries. This article aims to provide an overview of current knowledge on the subject with an assessment of the quality of the evidence in order to allow anaesthesiologists all over Europe to integrate - wherever possible - this knowledge into daily patient care. The Guidelines Committee of the ESA formed a task force comprising members of the previous task force, members of ESA scientific subcommittees and an open call for volunteers was made to all individual active members of the ESA and national societies. Electronic databases were searched from July 2010 (end of the literature search of the previous ESA guidelines on pre-operative evaluation) to May 2016 without language restrictions. A total of 34066 abtracts were screened from which 2536 were included for further analysis. Relevant systematic reviews with meta-analyses, randomised controlled trials, cohort studies, case-control studies and cross-sectional surveys were selected. The Grading of Recommendations Assessment, Development and Evaluation (GRADE) system was used to assess the level of evidence and to grade recommendations. The final draft guideline was posted on the ESA website for 4 weeks and the link was sent to all ESA members, individual or national (thus including most European national anaesthesia societies). Comments were collated and the guidelines amended as appropriate. When the final draft was complete, the Guidelines Committee and ESA Board ratified the guidelines