Porto-Splenic veins thrombosis and Budd-Chiari syndrome in a patient with essential thrombocythemia – is it always the myeloproliferative neoplasm to blame?

Essential Thrombocythemia (ET) is one of seven Myeloproliferative Neoplasms (MPNs), according to the latest WHO classification [1].&nbsp;</p

Acute Panmyelosis with Myelofibrosis: The Serpentine Road to Diagnosis

Acute Panmyelosis with Myelofibrosis (APMF) is an extremely rare disease. Lack of familiarity with this rare subtype of acute leukemia hinders the diagnosis [1]. It is characterized by an acute, rapidly progressive, fatal clinical course, peripheral blood cytopenias with minimum 20% blast and no detectable splenomegaly. APMF corresponds to less than 1% of the cases of Acute Myeloid Leukemia (AML) [2]. Histologically, APMF is characterized by a heavily fibrotic marrow, an increased number of immature hematopoietic elements (panmyelosis), and dysplastic population of megakaryocytes. The condition should be differentiated from other hematological neoplasms that present with fibrosis such as acute megakaryoblastic leukemia and myelodysplsia (MDS) with myelofibrosis. Dacrocytes are not present.</p

Sex Differences in Plasma MicroRNA Biomarkers of Early and Complicated Diabetes Mellitus in Israeli Arab and Jewish Patients

MicroRNAs play functional roles in the etiology of type 2 diabetes mellitus (T2DM) and complications, and extracellular microRNAs have attracted interest as potential biomarkers of these conditions. We aimed to identify a set of plasma microRNAs, which could serve as biomarkers of T2DM and complications in a mixed Israeli Arab/Jewish patient sample. Subjects included 30 healthy volunteers, 29 early-stage T2DM patients, and 29 late-stage T2DM patients with renal and/or vascular complications. RNA was isolated from plasma, and the levels of 12 candidate microRNAs were measured by quantitative reverse transcription and polymerase chain reaction (qRT-PCR). MicroRNA levels were compared between the groups and correlated to clinical measurements, followed by stepwise regression analysis and discriminant analysis. Plasma miR-486-3p and miR-423 were respectively up- and down-regulated in T2DM patients compared to healthy controls. MiR-28-3p and miR-423 were up-regulated in patients with complicated T2DM compared to early T2DM, while miR-486-3p was down-regulated. Combined, four microRNAs (miR-146a-5p, miR-16-2-3p, miR-126-5p, and miR-30d) could distinguish early from complicated T2DM with 77% accuracy and 79% sensitivity. In male patients only, the same microRNAs, with the addition of miR-423, could distinguish early from complicated T2DM with 83.3% accuracy. Furthermore, plasma microRNA levels showed significant correlations with clinical measurements, and these differed between men and women. Additionally, miR-183-5p levels differed significantly between the ethnic groups. Our study identified a panel of specific plasma microRNAs which can serve as biomarkers of T2DM and its complications and emphasizes the importance of sex differences in their clinical application

Cell-free IgG-aggregates in plasma of patients with chronic lymphocytic leukemia cause chronic activation of the classical complement pathway.

Therapy regimens for Chronic lymphocytic leukemia (CLL) commonly include chemotherapy and immunotherapy, which act through complement-mediated-cytotoxicity (CDC) and other mechanisms. CDC depends on several factors, including the availability and activity of the complement classical pathway (CP). Recently, a significant decrease in CP activity was shown to be associated with an immunoglobulin-C5a complex (Ig-C5a) and other markers of chronic CP activation in 40% of the patients. The study focused on the involvement of IgG-hexamers, an established CP activator, in the mechanism of chronic CP activation in CLL. Sera from 51 naïve CLL patients and 20 normal controls were collected. CP and alternative pathway (AP) activities were followed by the complement activity marker sC5b-9. Serum high molecular weight (HMW) proteins were collected by gel-filtration chromatography and their complement activation capacity was assessed. The levels of IgM, another established CP activator, were measured. Data were associated with the presence of Ig-C5a. Baseline levels of activation markers negatively correlated with CP and the AP activities, supporting chronic complement activation. In patients with Ig-C5a, HMW proteins that are not IgM, activated the complement. HMW proteins were identified as IgG-aggregates by affinity binding assays and Western blot analysis. The data indicate chronic CP activation, mediated by cell-free IgG-hexamers as a cause of decreased CP activity in part of the CLL population. This mechanism may affect immunotherapy outcomes due to compromised CP activity and CDC

Reasons underlying the intention to vaccinate children aged 5-11 against COVID-19: A cross-sectional study of parents in Israel, November 2021

Vaccination is a key tool to mitigate impacts of the COVID-19 pandemic. In Israel, COVID-19 vaccines became available to adults in December 2020 and to 5–11-year-old children in November 2021. Ahead of the vaccine roll-out in children, we aimed to determine whether surveyed parents intended to vaccinate their children and describe reasons for their intentions. We collected information on parental socio-demographic characteristics, COVID-19 vaccine history, intention to vaccinate their children against COVID-19, and reasons for parental decisions using an anonymous online survey. We identified associations between parental characteristics and plans to vaccinate children using a logistic regression model and described reasons for intentions to vaccinate or not. Parental non-vaccination and having experienced major vaccination side effects were strongly associated with non-intention to vaccinate their children (OR 0.09 and 0.18 respectively, p < .001). Parents who were younger, lived in the socio-economically deprived periphery, and belonged to the Arab population had lower intentions to vaccinate their children. Reasons for non-intention to vaccinate included concerns about vaccine safety and efficacy (53%, 95%CI 50–56) and the belief that COVID-19 is a mild disease (73%, 95%CI 73–79), while a frequent motive for vaccination was the return to normal social and educational life (89%, 95%CI 87–91). Understanding rationales for COVID-19 vaccine rejection or acceptance, as well as parental demographic data, can pave the way for intentional educational campaigns to encourage not only vaccination against COVID-19, but also regular childhood vaccine programming

Additional file 1: of Physicians’ lack of knowledge - a possible reason for red blood cell transfusion overuse?

Blood transfusion questionnaire. (DOCX 14 kb

A C5a-Immunoglobulin complex in chronic lymphocytic leukemia patients is associated with decreased complement activity.

Chronic lymphocytic leukemia (CLL) is the most common adult leukemia in the Western world. The therapeutic approach to CLL includes chemotherapeutic regimens and immunotherapy. Complement-mediated cytotoxicity, which is one of the mechanisms activated by the therapeutic monoclonal antibodies, depends on the availability and activity of the complement (C) system. The aim was to study the structure of circulating C components and evaluate the importance of C5 structural integrity for C activity in CLL patients. Blood samples were collected from 40 naïve CLL patients and 15 normal controls (NC). The Western blot analysis showed abnormal C5 pattern in some CLL patients, while patterns of C3 and C4 were similar in all subjects. Levels of the C activation markers sC5b-9 and C5a were quantified before and after activation via the classical (CP) and alternative (AP) pathways. In patients with abnormal C5, basal levels of sC5b-9 and C5a were increased while activities of the CP and of the CP C5-convertase, the immediate C5-upstream complex, were decreased compared to NC and to patients with normal C5. The data indicate a link between CP activation and apparent C5 alterations in CLL. This provides a potential prognostic tool that may personalize therapy by identifying a sub-group of CLL patients who display an abnormal C5 pattern, high basal levels of sC5b-9 and C5a, and impaired CP activity, and are likely to be less responsive to immunotherapy due to compromised CP activity

Dedicated mass-casualty incident hospitals : An overview

Introduction Hospitals worldwide are preparing for mass casualty incidents (MCIs). The Major Incident Hospital in the Netherlands was constructed 25 years ago as a dedicated hospital for situations wherein a sudden increase in medical surge capacity is mandated to handle an MCI. Over the years, more initiatives of dedicated MCIs have arisen. Herein, we compared the MCI facilities from three countries considering the reasons for construction and the functionality. Methods Three dedicated mass casualty hospitals and one hospital with a largely fortified structure were compared. The centres were located in the Netherlands, Italy, and Israel. Between August 2015 and January 2016, structured interviews were conducted with representatives of the hospitals’ medical operations. The interviews focussed on general information regarding the need for MCI preparedness and scenarios that require preparation, reasons for construction, hospital missions, and the experiences gained including training. Results All dedicated MCI hospitals had a common policy wherein they sought to create normal work circumstances for the medical staff by using similar equipment and resources as in normal hospitals. The MCI hospitals’ designs differed substantially, as determined by the threats faced by the country. In Europe, these hospitals are designed as a solution to surge capacity and function as buffer hospitals offering readily available, short term, additional medical capacity to the local health care system. Israel faces constant threat from long-term conflicts; during the 2006 war, several hospitals suffered direct missile impacts. Therefore, Israeli MCI hospitals are designed to be fortified structures offering shelter against both conventional and non-conventional warfare and intended as a long-term solution during siege situations. Conclusion Several dedicated MCI hospitals are presently being constructed. During construction, the local circumstances should be taken into account to determine the functionality for both short-term solutions for surge capacity and as fortified structures to withstand under-siege situations

Dedicated mass-casualty incident hospitals: An overview

Introduction Hospitals worldwide are preparing for mass casualty incidents (MCIs). The Major Incident Hospital in the Netherlands was constructed 25 years ago as a dedicated hospital for situations wherein a sudden increase in medical surge capacity is mandated to handle an MCI. Over the years, more initiatives of dedicated MCIs have arisen. Herein, we compared the MCI facilities from three countries considering the reasons for construction and the functionality. Methods Three dedicated mass casualty hospitals and one hospital with a largely fortified structure were compared. The centres were located in the Netherlands, Italy, and Israel. Between August 2015 and January 2016, structured interviews were conducted with representatives of the hospitals\u2019 medical operations. The interviews focussed on general information regarding the need for MCI preparedness and scenarios that require preparation, reasons for construction, hospital missions, and the experiences gained including training. Results All dedicated MCI hospitals had a common policy wherein they sought to create normal work circumstances for the medical staff by using similar equipment and resources as in normal hospitals. The MCI hospitals\u2019 designs differed substantially, as determined by the threats faced by the country. In Europe, these hospitals are designed as a solution to surge capacity and function as buffer hospitals offering readily available, short term, additional medical capacity to the local health care system. Israel faces constant threat from long-term conflicts; during the 2006 war, several hospitals suffered direct missile impacts. Therefore, Israeli MCI hospitals are designed to be fortified structures offering shelter against both conventional and non-conventional warfare and intended as a long-term solution during siege situations. Conclusion Several dedicated MCI hospitals are presently being constructed. During construction, the local circumstances should be taken into account to determine the functionality for both short-term solutions for surge capacity and as fortified structures to withstand under-siege situations