Aleukemic bcr-abl positive granulocytic sarcoma

Granulocytic sarcoma (GS) can occur de novo or in association with intramedullary myeloid disorders. With the advent of sophisticated molecular detection techniques to detect diagnostic genes such as bcrabl, PML-RARAandCBFB/MYH11 in bone marrowor peripheral blood,many cases of the so called ‘primary’ GS are questionable. We report a case of primary GS where the tumor mass bcr-abl translocation was demonstrated by fluorescent in situ hybridization in which there was no evidence of chronic myeloid leukemia (CML). This is an important finding as it highlights the possibility that CML may present as a sole extramedullary form, and illustrates potential treatment by tyrosine kinase inhibitor

Outcome effectiveness of the severe sepsis resuscitation bundle with addition of lactate clearance as a bundle item: a multi-national evaluation

10.1186/cc10469Critical Care155R22

Label-free biophysical markers from whole blood microfluidic immune profiling reveals severe immune response signatures

10.1002/smll.202006123SMALL171

Asthma among adult patients presenting with dyspnea to the emergency department: An observational study

Introduction: Shortness of breath is a common presenting symptom to the emergency department (ED) that can arise from a myriad of possible diagnoses. Asthma is one of the major causes. Objective: The aim of this study was to describe the demographic features, clinical characteristics, management and outcomes of adults with an ED diagnosis of asthma who presented to an ED in the Asia Pacific region with a principal symptom of dyspnea. Methods: Planned sub‐study of patients with an ED diagnosis of asthma identified in the Asia, Australia and New Zealand Dyspnoea in Emergency Departments (AANZDEM) study. AANZDEM was a prospective cohort study conducted in 46 EDs in Australia, New Zealand, Singapore, Hong Kong and Malaysia over three 72 hour periods in May, August and October 2014. Primary outcomes were patient epidemiology, clinical features, treatment and outcomes (hospital length of stay (LOS) and mortality). Results: Of the 3044 patients with dyspnea, 387 (12.7%) patients had an ED diagnosis of asthma. The median age was 45 years, 60.1% were female, 16.1% were active or recent smokers and 30.4% arrived by ambulance. Inhaled bronchodilator therapy was initiated in 88.1% of patients, and 66.9% received both inhaled bronchodilators and systemic corticosteroids. After treatment in the ED, 65.4% were discharged. No death was reported. Conclusion: Asthma is common among patients presenting with a principal symptom of dyspnea in the ED of the Asia Pacific region. There was a suboptimal adherence to international guidelines on investigations and treatments of acute asthma exacerbations presenting an opportunity to improve the efficiency of care.Full Tex

The Outcome of HyperCVAD Combined with Alemtuzumab for the Treatment of Aggressive T-Cell and NK-Cell Neoplasms.

We report our experience in using six cycles of hyperCVAD in combination with alemtuzumab for the treatment of aggressive T-cell and NK/T-cell neoplasms. Seven females and six males with the median age of 41 (range 18–60) diagnosed with T-cell acute lymphoblastic lymphoma and peripheral T-cell and NK/T-cell neoplasms (nPTCL = 6, nT-cell ALL = 3, nNK/T-cell neoplasms = 4) from 2006 to 2008 were treated with alemtuzumab–hyperCVAD regimen. A total of nine patients (69%) responded to the regimen, with seven achieved complete remission and two achieved partial remission. The median progression free survival and overall survival duration among the responders with complete remission were 12.9 and 24.9 months respectively. The incidence of relapse among the responders was 44% and the overall survival rate was 23%. Only four (31%) patients completed the six cycles of alemtuzumab– hyperCVAD. Others were stopped earlier due to progressive disease (n = 2), cytomegalovirus (CMV) reactivation and/or disease (n = 3), death not due to disease (n = 2), and patient’s refusal to continue alemtuzumab (n = 2). The incidence of death not due to disease, CMV reactivation and recurrent CMV reactivation were 50, 50 and 17%, respectively. This study shows that alemtuzumab in combination with hyperCVAD regimen is a feasible regimen but with high toxicity. The toxicity might be reduced with the incorporation of filgrastim and use of valganciclovir as CMV prophylaxis

Validation of "Care of the Dying Evaluation" in Emergency Medicine (CODE-EM): pilot phase of end-of-life management protocol offered within emergency room (EMPOWER) study.

BackgroundAn increasing number of patients who present to emergency departments are at their end-of-life phase and have significant palliative care needs such as in symptom control for pain and dyspnoea. Evaluating quality of care provided is imperative, yet there is no suitable tool validated in the emergency and Asian settings. We aim to examine the face and construct validity, and reliability of a newly developed questionnaire, Care of the Dying Evaluation - Emergency Medicine, for measuring the quality of end-of-life care in an Asian emergency context.MethodsA mixed methods pilot study was conducted. Participants composed of the next-of-kin to thirty dying patients who presented to the emergency departments of three public hospitals in Singapore. Qualitative evaluation, using cognitive "think-aloud" interviews, and quantitative analysis were employed. Percentage agreement and κ statistic were measured to evaluate temporal stability of the questionnaire. Cronbach's α and item-total correlations were used to assess internal consistency within the constructs. Confirmatory factor analysis was performed for construct validity.ResultsAll participants reported clear understanding of the questionnaire with no ambiguity; a minority felt the questions caused emotional distress (7/30, 23.3%). The questions showed moderate to good test-retest reliability. Internal consistencies within the constructs were good for "ENVIRONMENT" and "CARE", and moderate for "COMMUNICATION". Factor loadings range from 0.40 to 0.99.ConclusionsThe Care of the Dying Evaluation - Emergency Medicine questionnaire may be valid and reliable for use in an Asian emergency setting. Our prospective multicentre study using this evaluation tool may provide more insight on the quality of care rendered to dying patients and identify areas for improvement.Trial registrationClinicalTrials.gov (NCT03906747)

Epidemiology, prehospital care and outcomes of patients arriving by ambulance with dyspnoea: An observational study

Background: This study aimed to determine epidemiology and outcome for patients presenting to emergency departments (ED) with shortness of breath who were transported by ambulance. Methods: This was a planned sub-study of a prospective, interrupted time series cohort study conducted at three time points in 2014 and which included consecutive adult patients presenting to the ED with dyspnoea as a main symptom. For this sub-study, additional inclusion criteria were presentation to an ED in Australia or New Zealand and transport by ambulance. The primary outcomes of interest are the epidemiology and outcome of these patients. Analysis was by descriptive statistics and comparisons of proportions. Results: One thousand seven patients met inclusion criteria. Median age was 74 years (IQR 61-68) and 46.1 % were male. There was a high rate of co-morbidity and chronic medication use. The most common ED diagnoses were lower respiratory tract infection (including pneumonia, 22.7 %), cardiac failure (20.5%) and exacerbation of chronic obstructive pulmonary disease (19.7 %). ED disposition was hospital admission (including ICU) for 76.4 %, ICU admission for 5.6 % and death in ED in 0.9 %. Overall in-hospital mortality among admitted patients was 6.5 %. Discussion: Patients transported by ambulance with shortness of breath make up a significant proportion of ambulance caseload and have high comorbidity and high hospital admission rate. In this study, >60 % were accounted for by patients with heart failure, lower respiratory tract infection or COPD, but there were a wide range of diagnoses. This has implications for service planning, models of care and paramedic training. Conclusion: This study shows that patients transported to hospital by ambulance with shortness of breath are a complex and seriously ill group with a broad range of diagnoses. Understanding the characteristics of these patients, the range of diagnoses and their outcome can help inform training and planning of services

Blood transcriptome responses in patients correlate with severity of COVID-19 disease

BackgroundCoronavirus disease 2019 (COVID-19) is an infectious disease caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2). Infected individuals display a wide spectrum of disease severity, as defined by the World Health Organization (WHO). One of the main factors underlying this heterogeneity is the host immune response, with severe COVID-19 often associated with a hyperinflammatory state.AimOur current study aimed to pinpoint the specific genes and pathways underlying differences in the disease spectrum and outcomes observed, through in-depth analyses of whole blood transcriptomics in a large cohort of COVID-19 participants.ResultsAll WHO severity levels were well represented and mild and severe disease displaying distinct gene expression profiles. WHO severity levels 1-4 were grouped as mild disease, and signatures from these participants were different from those with WHO severity levels 6-9 classified as severe disease. Severity level 5 (moderate cases) presented a unique transitional gene signature between severity levels 2-4 (mild/moderate) and 6-9 (severe) and hence might represent the turning point for better or worse disease outcome. Gene expression changes are very distinct when comparing mild/moderate or severe cases to healthy controls. In particular, we demonstrated the hallmark down-regulation of adaptive immune response pathways and activation of neutrophil pathways in severe compared to mild/moderate cases, as well as activation of blood coagulation pathways.ConclusionsOur data revealed discrete gene signatures associated with mild, moderate, and severe COVID-19 identifying valuable candidates for future biomarker discovery