Mechanisms of leukemic transformation in congenital neutropenia
Purpose of review
The development of a myelodysplastic syndrome (MDS) or acute myeloid leukemia (AML) in patients with congenital neutropenia is now the major cause of mortality. Treatment options are limited and there are no effective prevention strategies. This review focuses on mechanisms of leukemic transformation in severe congenital neutropenia (SCN) and Shwachman–Diamond syndrome (SDS), the two most common types of congenital neutropenia.
Recent findings
AML/MDS that develops in the setting of congenital neutropenia has distinct molecular features. Clonal hematopoiesis because of TP53 mutations is seen in nea...
Source: Current Opinion in Hematology - November 28, 2018 Category: Hematology Tags: MYELOID BIOLOGY: Edited by David C. Dale Source Type: research
The role of CXCR2 in acute inflammatory responses and its antagonists as anti-inflammatory therapeutics
Purpose of review
CXCR2 is key stimulant of immune cell migration and recruitment, especially of neutrophils. Alleviating excessive neutrophil accumulation and infiltration could prevent prolonged tissue damage in inflammatory disorders. This review focuses on recent advances in our understanding of the role of CXCR2 in regulating neutrophil migration and the use of CXCR2 antagonists for therapeutic benefit in inflammatory disorders.
Recent findings
Recent studies have provided new insights into how CXCR2 signaling regulates hematopoietic cell mobilization and function in both health and disease. We also summarize sev...
Source: Current Opinion in Hematology - November 28, 2018 Category: Hematology Tags: MYELOID BIOLOGY: Edited by David C. Dale Source Type: research
The clinical consequences of neutrophil priming
Purpose of review
Neutrophils priming has been long studied in vitro. Recent studies describe it in vivo. In pathophysiological conditions, complex, heterogeneous characteristics of priming are described in the last few years.
Recent findings
Priming can occur systemically when insults such as sepsis or trauma result in an array of circulating mediators and circulating primed neutrophils seem to exert detrimental effects either directly, or indirectly by interacting with other cells, thereby contributing to the development of organ dysfunction. Local priming of neutrophils augments their ability to clear infection, bu...
Source: Current Opinion in Hematology - November 28, 2018 Category: Hematology Tags: MYELOID BIOLOGY: Edited by David C. Dale Source Type: research
Neutropenia in glycogen storage disease Ib: outcomes for patients treated with granulocyte colony-stimulating factor
Purpose of review
Glycogen storage disease Ib (GSD Ib) is characterized by hepatomegaly, hypoglycemia, neutropenia, enterocolitis and recurrent bacterial infections. It is attributable to mutations in G6PT1, the gene for the glucose-6-phosphate transporter responsible for transport of glucose into the endoplasmic reticulum. Neutropenia in GSD Ib is now frequently treated with granulocyte colony-stimulating factor (G-CSF). We formed a cooperative group to review outcomes of the long-term treatment of GSD Ib patients treated with G-CSF.
Recent findings
The study enrolled 103 patients (48 men and 55 women), including 47 ...
Source: Current Opinion in Hematology - November 28, 2018 Category: Hematology Tags: MYELOID BIOLOGY: Edited by David C. Dale Source Type: research
Neutropenia in Barth syndrome: characteristics, risks, and management
Purpose of review
Barth syndrome (BTHS) is an X-linked disease characterized by defective remodeling of phospholipid side chains in mitochondrial membranes. Major features include neutropenia, dilated cardiomyopathy, motor delay and proximal myopathy, feeding problems, and constitutional growth delay. We conducted this review of neutropenia in BTHS to aid in the diagnosis of this disease, and to improve understanding of both the consequences of neutropenia and the benefits of treatment with granulocyte colony-stimulating factor (G-CSF).
Recent findings
In 88 patients with BTHS, neutropenia, that is, at least one count...
Source: Current Opinion in Hematology - November 28, 2018 Category: Hematology Tags: MYELOID BIOLOGY: Edited by David C. Dale Source Type: research
Editorial: Update in myeloid biology
No abstract available (Source: Current Opinion in Hematology)
Source: Current Opinion in Hematology - November 28, 2018 Category: Hematology Tags: MYELOID BIOLOGY: Edited by David C. Dale Source Type: research
Editorial introductions
No abstract available (Source: Current Opinion in Hematology)
Source: Current Opinion in Hematology - November 28, 2018 Category: Hematology Tags: EDITORIAL INTRODUCTIONS Source Type: research
The role of genomics in transfusion medicine
Purpose of review
To summarize recent advances in red blood cell (RBC) blood group genotyping, with an emphasis on advances in the use of NGS next generation sequencing (NGS) to detect clinically relevant blood group gene variation.
Recent findings
Genetic information is useful in predicting RBC blood group antigen expression in several clinical contexts, particularly, for patients at high-risk for allosensitization, such as multiple transfused patients. Blood group antigen expression is directed by DNA variants affecting multiply genes. With over 300 known antigens, NGS offers the attractive prospect of comprehensive...
Source: Current Opinion in Hematology - October 5, 2018 Category: Hematology Tags: TRANSFUSION MEDICINE AND IMMUNOHEMATOLOGY: Edited by Steven L. Spitalnik Source Type: research
Towards increasing shelf life and haemostatic potency of stored platelet concentrates
Purpose of review
Platelet transfusion is a widely used therapy in treating or preventing bleeding and haemorrhage in patients with thrombocytopenia or trauma. Compared with the relative ease of platelet transfusion, current practice for the storage of platelets is inefficient, costly and relatively unsafe, with platelets stored at room temperature (RT) for upto 5–7 days.
Recent findings
During storage, especially at cold temperatures, platelets undergo progressive and deleterious changes, collectively termed the ‘platelet storage lesion’, which decrease their haemostatic function and posttransfusion survival. R...
Source: Current Opinion in Hematology - October 5, 2018 Category: Hematology Tags: TRANSFUSION MEDICINE AND IMMUNOHEMATOLOGY: Edited by Steven L. Spitalnik Source Type: research
The clinical impact of glucose-6-phosphate dehydrogenase deficiency in patients with sickle cell disease
Purpose of review
Glucose-6-phosphate dehydrogenase (G6PD) deficiency and sickle cell disease (SCD) cause hemolysis, often occurring in individuals of African descent. These disorders co-occur frequently, and possibly interact, altering clinical outcomes in SCD. However, epidemiological investigations of SCD with G6PD deficiency have produced variable results. This contribution reviews the available data about the interaction of G6PD deficiency and SCD.
Recent findings
Overall, G6PD deficiency contributes few, if any, effects to laboratory values and clinical outcomes in SCD patients, but may impact transfusion effica...
Source: Current Opinion in Hematology - October 5, 2018 Category: Hematology Tags: TRANSFUSION MEDICINE AND IMMUNOHEMATOLOGY: Edited by Steven L. Spitalnik Source Type: research
Restoring antiviral immunity with adoptive transfer of ex-vivo generated T cells
Purpose of review
Latent viruses such as cytomegalovirus (CMV), Epstein–Barr virus (EBV) and adenovirus (ADV) often reactivate in immunocompromised patients, contributing to poor clinical outcomes. A rapid reconstitution of antiviral responses via adoptive transfer of virus-specific T cells (VSTs) can prevent or eradicate even refractory infections. Here, we evaluate this strategy and the associated methodological, manufacturing and clinical advances.
Recent findings
From the early pioneering but cumbersome efforts to isolate CMV-specific T cell clones, new approaches and techniques have been developed to provide qu...
Source: Current Opinion in Hematology - October 5, 2018 Category: Hematology Tags: TRANSFUSION MEDICINE AND IMMUNOHEMATOLOGY: Edited by Steven L. Spitalnik Source Type: research
‘Massive transfusion protocols and the use of tranexamic acid’
Purpose of review
We review recent articles pertaining to the use of tranexamic acid (TXA) in populations at risk for massive transfusion. Although there are no recent studies that specifically examine the use of TXA in massive transfusion protocols (MTPs), there are a few studies with subgroups of massive transfusion patients.
Recent findings
In recent years, many publications have discussed outcomes and safety associated with the addition of TXA to treatment plans for bleeding pediatric, trauma, and postpartum hemorrhage patients. In general, TXA appears to decrease mortality and transfusion requirements.
Summary
...
Source: Current Opinion in Hematology - October 5, 2018 Category: Hematology Tags: TRANSFUSION MEDICINE AND IMMUNOHEMATOLOGY: Edited by Steven L. Spitalnik Source Type: research
Murine models of autoimmune hemolytic anemia
Purpose of review
Pathogenic autoantibodies directed against red blood cells (RBCs) may lead to autoimmune hemolytic anemia (AIHA), a severe and sometimes fatal disease. Much of what is known about the etiology and pathogenesis of AIHA has been learned from observations made in human patients and murine models, but many questions remain; importantly, it is still unclear why some people generate RBC-specific autoantibodies. The combination of technological advancements applied to existing models and the development of new AIHA murine models will continue to provide considerable insight into the initiation of AIHA and prov...
Source: Current Opinion in Hematology - October 5, 2018 Category: Hematology Tags: TRANSFUSION MEDICINE AND IMMUNOHEMATOLOGY: Edited by Steven L. Spitalnik Source Type: research
To transfuse or not transfuse: an intensive appraisal of red blood cell transfusions in the ICU
Purpose of review
This review is a critical appraisal of the current data comparing restrictive vs. liberal transfusion strategies for patients who are critically ill in ICUs. We focus on four subsets of critically ill patients: pediatric patients, patients with gastrointestinal bleeds, septic patients and patients undergoing cardiac surgery.
Recent findings
Almost a decade after the TRICC trial, a randomized trial showing the safety of a restrictive transfusion threshold in critically ill patients, four large randomized controlled trials have shown that a restrictive transfusion strategy is safe in pediatric critical...
Source: Current Opinion in Hematology - October 5, 2018 Category: Hematology Tags: TRANSFUSION MEDICINE AND IMMUNOHEMATOLOGY: Edited by Steven L. Spitalnik Source Type: research
Delayed haemolytic and serologic transfusion reactions: pathophysiology, treatment and prevention
Purpose of review
The aim of this study was to summarize the basic epidemiology, pathophysiology and management of delayed serologic and delayed haemolytic transfusion reactions (DHTRs), as well as recent developments in our understanding of these adverse events.
Recent findings
Several studies have identified risk factors for DHTRs, including high alloantibody evanescence rates among both general patient groups and those with sickle cell disease (SCD). Antibody detection is also hampered by the phenomenon of transfusion record fragmentation. There have also been enhancements in understanding of what may contribute to...
Source: Current Opinion in Hematology - October 5, 2018 Category: Hematology Tags: TRANSFUSION MEDICINE AND IMMUNOHEMATOLOGY: Edited by Steven L. Spitalnik Source Type: research
