The 2016 revision of WHO classification of myeloproliferative neoplasms: Clinical and molecular advances
Clinical evidence supports the need of changing the diagnostic criteria of the 2008 updated WHO classification for polycythemia vera (PV), essential thrombocythemia (ET) and primary myelofibrosis (PMF). In JAK2-mutated patients who show characteristic bone marrow (BM) morphology, clinical studies demonstrated that a hemoglobin level of 16.5g/dL in men and 16.0g/dl for women or a hematocrit value of 49% in men and 48% in women are the optimal cut off levels for distinguishing JAK2-mutated ET from “masked/prodromal” PV. (Source: Blood Reviews)
Source: Blood Reviews - June 10, 2016 Category: Hematology Authors: T. Barbui, J. Thiele, H. Gisslinger, G. Finazzi, A.M. Vannucchi, A. Tefferi Tags: REVIEW Source Type: research
Could stem cells be the future therapy for sepsis?
The severity and threat of sepsis is well known, and despite several decades of research, the mortality continues to be high. Stem cells have great potential to be used in various clinical disorders. The innate ability of stem cells such as pluripotency, self-renewal makes them potential agents for therapeutic intervention. The pathophysiology of sepsis is a plethora of complex mechanisms which include the initial microbial infection, followed by “cytokine storm,” endothelial dysfunction, coagulation cascade, and the late phase of apoptosis and immune paralysis which ultimately results in multiple organ dysfunction. (S...
Source: Blood Reviews - June 4, 2016 Category: Hematology Authors: S. Manoj Kumar Kingsley, B. Vishnu Bhat Tags: REVIEW Source Type: research
Could stem cells be the future therapy for sepsis?
The severity and threat of sepsis is well known and despite several decades of research the mortality continues to be high. Stem cells have great potential to be used in various clinical disorders. The innate ability of stem cells such as pluripotency, self-renewal makes them potential agents for therapeutic intervention. The pathophysiology of sepsis is a plethora of complex mechanisms which include the initial microbial infection, followed by ‘cytokine storm’, endothelial dysfunction, coagulation cascade and the late phase of apoptosis and immune paralysis which ultimately results in multiple organ dysfunction. (Source: Blood Reviews)
Source: Blood Reviews - June 4, 2016 Category: Hematology Authors: S. Manoj Kumar Kingsley, B. Vishnu Bhat Tags: REVIEW Source Type: research
Hyperferritinemia and iron metabolism in Gaucher disease: Potential pathophysiological implications
Gaucher disease (GD) is characterized by large amounts of lipid-storing macrophages and is associated with accumulation of iron. High levels of ferritin are a hallmark of the disease. The precise mechanism underlying the changes in iron metabolism has not been elucidated. A systematic search was conducted to summarize available evidence from the literature on iron metabolism in GD and its potential pathophysiological implications. We conclude that in GD, a chronic low grade inflammation state can lead to high ferritin levels and increased hepcidin transcription with subsequent trapping of ferritin in macrophages. (Source: Blood Reviews)
Source: Blood Reviews - May 26, 2016 Category: Hematology Authors: Martine Regenboog, Andr é B.P. van Kuilenburg, Joanne Verheij, Dorine W. Swinkels, Carla E.M. Hollak Tags: REVIEW Source Type: research
Hyperferritinemia and iron metabolism in Gaucher Disease: potential pathophysiological implications
Gaucher Disease (GD) is characterized by large amounts of lipid-storing macrophages and is associated with accumulation of iron. High levels of ferritin are a hallmark of the disease. The precise mechanism underlying the changes in iron metabolism has not been elucidated. A systematic search was conducted to summarize available evidence from the literature on iron metabolism in GD and its potential pathophysiological implications. We conclude that in GD, a chronic low grade inflammation state can lead to high ferritin levels and increased hepcidin transcription with subsequent trapping of ferritin in macrophages. (Source: Blood Reviews)
Source: Blood Reviews - May 26, 2016 Category: Hematology Authors: Martine Regenboog, André B.P. van Kuilenburg, Joanne Verheij, Dorine W. Swinkels, Carla E.M. Hollak Tags: REVIEW Source Type: research
Hemophagocytic syndromes (HPSs) including hemophagocytic lymphohistiocytosis (HLH) in adults: A systematic scoping review
Most knowledge of hemophagocytic syndromes (HPSs) including hemophagocytic lymphohistiocytosis (HLH) is derived from pediatric studies; literature on adult HPS/HLH predominantly consists of small retrospective studies with clinical and methodological heterogeneity. The aims of this systematic scoping review were to provide an overview of existing literature on adult HPS/HLH, describe current practices in diagnosis and treatment, and propose priorities for future research. Articles from Ovid Medline, Embase and Pubmed (1975 –2015) describing 10 or more unique adults (age > 15years) with HPS/HLH were included. (Source: Blood Reviews)
Source: Blood Reviews - May 18, 2016 Category: Hematology Authors: Anna Hayden, Sujin Park, Dean Giustini, Agnes Y.Y. Lee, Luke Y.C. Chen Tags: REVIEW Source Type: research
The contribution of different adipose tissue depots to plasma plasminogen activator inhibitor-1 (PAI-1) levels
Increased plasma plasminogen activator inhibitor-1 (PAI-1) level is considered a mechanistic pathway through which obesity contributes to increased cardiovascular disease risk. Abdominal adipose tissue specifically, is a major PAI-1 source with visceral adipose tissue (VAT), an ectopic fat depot, generally considered to produce more PAI-1 than subcutaneous adipose tissue. However, this does not necessarily lead to increased plasma PAI-1 levels. This review provides an overview of studies investigating the association between body fat distribution and plasma PAI-1 levels. (Source: Blood Reviews)
Source: Blood Reviews - May 18, 2016 Category: Hematology Authors: Sunelle A. Barnard, Marlien Pieters, Zelda De Lange Tags: REVIEW Source Type: research
Hemophagocytic syndromes (HPSs) including hemophagocytic lymphohistiocytosis (HLH) in adults: A systematic scoping review
Most knowledge of hemophagocytic syndromes (HPSs) including hemophagocytic lymphohistiocytosis (HLH) is derived from pediatric studies; literature on adult HPS/HLH predominantly consists of small retrospective studies with clinical and methodological heterogeneity. The aims of this systematic scoping review were to provide an overview of existing literature on adult HPS/HLH, describe current practices in diagnosis and treatment, and propose priorities for future research. Articles from Ovid Medline, Embase and Pubmed (1975–2015) describing 10 or more unique adults (age>15years) with HPS/HLH were included. (Source: Blood Reviews)
Source: Blood Reviews - May 18, 2016 Category: Hematology Authors: Anna Hayden, Sujin Park, Dean Giustini, Agnes Y.Y. Lee, Luke Y.C. Chen Tags: REVIEW Source Type: research
Hemophagocytic syndromes (HPS) including Hemophagocytic Lymphohistiocytosis (HLH) in Adults: A Systematic Scoping Review
Most knowledge of hemophagocytic syndromes (HPS) including hemophagocytic lymphohistiocytosis (HLH) is derived from pediatric studies; literature on adult HPS/HLH predominantly consists of small retrospective studies with clinical and methodological heterogeneity. The aims of this systematic scoping review were to provide an overview of existing literature on adult HPS/HLH, describe current practices in diagnosis and treatment, and propose priorities for future research. Articles from Ovid Medline, Embase and Pubmed (1975–2015) describing 10 or more unique adults (age>15years) with HPS/HLH were included. (Source: Blood Reviews)
Source: Blood Reviews - May 18, 2016 Category: Hematology Authors: Anna Hayden, Sujin Park, Dean Giustini, Agnes Y.Y. Lee, Luke Y.C. Chen Tags: REVIEW Source Type: research
The contribution of different adipose tissue depots to plasma plasminogen activator inhibitor-1 (PAI-1) levels
Increased plasma plasminogen activator inhibitor-1 (PAI-1) level is considered a mechanistic pathway through which obesity contributes to increased cardiovascular disease risk. Abdominal adipose tissue specifically, is a major PAI-1 source with visceral adipose tissue (VAT), an ectopic fat depot, generally considered to produce more PAI-1 than subcutaneous adipose tissue. This does however, not necessarily lead to increased plasma PAI-1 levels. This review provides an overview of studies investigating the association between body fat distribution and plasma PAI-1 levels. (Source: Blood Reviews)
Source: Blood Reviews - May 18, 2016 Category: Hematology Authors: Sunelle A. Barnard, Marlien Pieters, Zelda De Lange Tags: REVIEW Source Type: research
Musculo-skeletal manifestations of haemophilia
Haemophilia left untreated or treated on demand destroys the joints at a very young age. Primary haematological prophylaxis, currently the gold standard for the treatment of haemophilia, is not completely effective. Moreover, it is only available for 25 –30% of patients worldwide. Advances in haematology, combined with the advances in orthopaedic surgery and other disciplines (physical medicine and rehabilitation, physiotherapy, specialised nursing, etc.), have made it possible to improve the musculo-skeletal manifestations of haemophilia in thes e patients through orthopaedic surgical interventions. (Source: Blood Reviews)
Source: Blood Reviews - May 3, 2016 Category: Hematology Authors: E. Carlos Rodriguez-Merchan Tags: REVIEW Source Type: research
Musculo-skeletal manifestations of haemophilia
Haemophilia left untreated or treated on demand destroys the joints at a very young age. Primary haematological prophylaxis, currently the gold standard for the treatment of haemophilia, is not completely effective. Moreover, it is only available for 25-30% of patients worldwide. Advances in haematology, combined with the advances in orthopaedic surgery and other disciplines (physical medicine and rehabilitation, physiotherapy, specialised nursing, etc.), have made it possible to improve the musculo-skeletal manifestations of haemophilia in these patients through orthopaedic surgical interventions. (Source: Blood Reviews)
Source: Blood Reviews - May 3, 2016 Category: Hematology Authors: E. Carlos Rodriguez-Merchan Tags: Review Source Type: research
Radioimmunotherapy-based conditioning for hematopoietic stem cell transplantation: Another step forward
This article is intended to provide a summary of the basic elements of this approach as well as a concise and up-to-date scoping review of the studies that evaluated the safety and efficacy of integrating radioimmunotherapy in the conditioning regimens. (Source: Blood Reviews)
Source: Blood Reviews - May 1, 2016 Category: Hematology Authors: Alaa M. Ali, Farrokh Dehdashti, John F. DiPersio, Amanda F. Cashen Tags: REVIEW Source Type: research
