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Expert Review of Hematology

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https://www.readbyqxmd.com/read/28914569/mutations-in-myeloproliferative-neoplasms-their-significance-and-clinical-use
#1
Fiorella Schischlik, Robert Kralovics
Clonal hematologic diseases of the blood such as polycythemia vera, essential thrombocythemia and primary myelofibrosis belong to the BCR-ABL negative Myeloproliferative Neoplasms (MPN). These diseases are characterized by clonal expansion of hematopoietic precursor cells followed by increased production of differentiated cells of the myeloid lineage. Initiation of clonal hematopoiesis, formation of a clinical phenotype as well as disease progression form part of MPN disease evolution. The disease is driven by acquired somatic mutations in critical pathways such as cytokine signaling, epigenetic regulation, RNA splicing, and transcription factor signaling...
September 15, 2017: Expert Review of Hematology
https://www.readbyqxmd.com/read/28905692/the-emerging-challenge-of-optimal-blood-pressure-management-and-hypertensive-syndromes-in-pregnant-women-with-sickle-cell-disease-a-review
#2
Nabilah F Lari, Michael R DeBaun, Samuel A Oppong
Sickle cell disease (SCD) is the most common hemoglobinopathy, affecting a considerable proportion of black populations of African origin, Middle East and in the Indian sub-continent. Women with SCD are more likely to experience adverse pregnancy and delivery outcomes. Hypertensive diseases in pregnancy such as preeclampsia and eclampsia are more common in women with sickle cell disease. Areas covered: This review examined the influence of hypertension and SCD in pregnancy, and provides the preliminary evidence that the traditional systolic and diastolic blood pressure thresholds for hypertensive disorders such as pre-eclampsia and eclampsia may require reassessment in pregnant women with SCD...
September 14, 2017: Expert Review of Hematology
https://www.readbyqxmd.com/read/28901221/direct-oral-anticoagulant-reversal-how-when-and-issues-faced
#3
Mikhail S Dzeshka, Daniele Pastori, Gregory Y H Lip
The number of atrial fibrillation (AF) patients requiring thrombo-prophylaxis with oral anticoagulation is greatly increasing. The introduction of non-vitamin K oral anticoagulants (NOACs) in addition to standard therapy with dose-adjusted warfarin has increased the therapeutic options for AF patients. Despite a generally better safety profile of the NOACs, the risk of major bleedings still persists, and the management of serious bleeding is a clinical challenge. Areas covered: In the current review, risk of major bleeding in patients taking NOACs and general approaches to manage bleeding depending on severity, with a particular focus on specific reversal agents, are discussed...
September 13, 2017: Expert Review of Hematology
https://www.readbyqxmd.com/read/28891355/fertility-challenges-for-women-with-sickle-cell-disease
#4
Djamila L Ghafuri, Sarah-Jo Stimpson, Melissa E Day, Andra James, Michael R DeBaun, Deva Sharma
Sickle cell disease (SCD) represents one of the most common monogenic blood disorders worldwide, with an incidence of over 300,000 newborns affected per year. Reproductive challenges for men and women with SCD have been previously reviewed; however, evidence-based strategies to prevent and manage infertility and increase fecundity are lacking in women with SCD, which is one of the most important factors for quality of life. Areas covered: This review article summarizes the known risk factors for infertility, low fecundity, and premature menopause related to SCD...
September 11, 2017: Expert Review of Hematology
https://www.readbyqxmd.com/read/28885063/treatment-free-interval-as-a-metric-of-patient-experience-and-a-health-outcome-of-value-for-advanced-multiple-myeloma-the-case-for-the-histone-deacetylase-inhibitor-panobinostat-a-next-generation-novel-agent
#5
Paul Richardson, Anuja Roy, Acharyya Suddhasatta, Ashok Panneerselvam, Estella Mendelson, Andreas Günther, Sagar Lonial, Hermann Einsele
BACKGROUND: Patients with relapsed or relapsed/refractory multiple myeloma (RRMM) face poor treatment options by the time third-line therapy is required, despite advances in overall survival in recent years. Treatment free interval (TFI) and opportunities to maintain quality of life (QoL) have been cited as additional measures of efficacy that can be utilized in personalized treatment decisions. METHODS: The clinical health outcomes data from PANORAMA-1, the pivotal phase-3 trial comparing panobinostat-bortezomib-dexamethasone (PAN-BTZ-DEX) with placebo (PBO)-BTZ-DEX in RRMM patients treated with 1 to 3 prior regimens, retrospectively assessed TFI as a health outcome measure and metric of patient treatment experience relevant to the RRMM population...
September 8, 2017: Expert Review of Hematology
https://www.readbyqxmd.com/read/28832236/icus-ccus-chip-basics-beyond
#6
Mili Jain, Anil Tripathi
Patients presenting with idiopathic cytopenia with non-diagnostic marrow morphology and a normal karyotype pose a diagnostic and therapeutic challenge. Additional diagnostic information from mutation analysis could provide important clinical insights. However, one has to be cautious during such diagnostic interpretations in view of the recent documentation of clonal somatic mutations in healthy elder individuals. Whether to regard clonality synonymous with malignant proliferation or a manifestation of ageing process is to be judged carefully...
September 1, 2017: Expert Review of Hematology
https://www.readbyqxmd.com/read/28829203/gender-related-issues-in-thrombosis-and-hemostasis
#7
Anne-Mette Hvas, Emmanuel J Favaloro
Many aspects of hemostasis, both primary and secondary, as well as fibrinolysis display sex differences. From a clinical viewpoint, certain differential phenotypic presentations clearly arise within various disorders of thrombosis and hemostasis. Areas covered: The present mini-review summarizes selected clinical entities where sex differences are reflected in both frequency and clinical presentation of hemostasis disorders. Sex differences are discussed within the settings of cardiovascular disease, including coronary artery disease and ischemic stroke, venous thromboembolism and inherited bleeding disorders...
August 30, 2017: Expert Review of Hematology
https://www.readbyqxmd.com/read/28814164/cpx-351-in-acute-myeloid-leukemia-can-a-new-formulation-maximize-the-efficacy-of-old-compounds
#8
Claudia Brunetti, Luisa Anelli, Antonella Zagaria, Giorgina Specchia, Francesco Albano
The management of Acute Myeloid Leukemia (AML) (with the exception of acute promyelocytic leukemia) has remained largely unchanged over the past 40 years. In particular, patients defined as high-risk, according to the 2017 European Leukemia Net recommendations, represent a subgroup with poor response to current therapies that are frequently associated with high-grade toxicity and potentially fatal complications. Areas covered: Preliminary results from an ongoing phase III clinical trial suggest that CPX-351 could represent an interesting treatment option in both induction and 'bridge-to-transplant' settings...
August 24, 2017: Expert Review of Hematology
https://www.readbyqxmd.com/read/28817980/pathways-to-pulmonary-hypertension-in-sickle-cell-disease-the-search-for-prevention-and-early-intervention
#9
Natalie R Shilo, Claudia R Morris
Pulmonary hypertension (PH) develops in a significant number of patients with sickle cell disease (SCD), resulting in increased morbidity and mortality. This review focuses on PH pathophysiology, risk stratification, and new recommendations for screening and treatment for patients with SCD. Areas covered: An extensive PubMed literature search was performed. While the pathophysiology of PH in SCD is yet to be fully deciphered, it is known that the etiology is multifactorial; hemolysis, hypercoagulability, hypoxemia, ischemic-reperfusion injury, oxidative stress, and genetic susceptibility all contribute in varying degrees to endothelial dysfunction...
August 23, 2017: Expert Review of Hematology
https://www.readbyqxmd.com/read/28828900/advances-in-fertility-preservation-of-female-patients-with-hematological-malignancies
#10
Mahmoud Salama, Vladimir Isachenko, Evgenia Isachenko, Gohar Rahimi, Peter Mallmann
The most common forms of hematological malignancies that occur in female reproductive years are lymphoma and leukemia. Areas covered: Several aggressive gonadotoxic regimens such as alkylating chemotherapy and total body irradiation are used frequently in treatment of lymphoma and leukemia leading to subsequent iatrogenic premature ovarian failure and fertility loss. In such cases, female fertility preservation options should be offered in advance. Expert commentary: In order to preserve fertility of young women and girls with lymphoma and leukemia, several established, experimental, and debatable options can be offered before starting chemotherapy and radiotherapy...
August 22, 2017: Expert Review of Hematology
https://www.readbyqxmd.com/read/28799436/immunotherapy-based-approaches-in-myelofibrosis
#11
Lucia Masarova, Srdan Verstovsek, Hagop Kantarjian, Naval Daver
Aberrant regulation of the immune system with up-regulation of pro-inflammatory cytokines contributes to disease pathophysiology in myelofibrosis (MF). Therapeutic options for MF associated anemia, thrombocytopenia, and bone marrow fibrosis remain limited. Areas covered: This review focuses on immune based therapies in MF, including immunomodulatory imide drugs (IMiDs), interferons, monoclonal antibodies and targeted agents (SL-401), and checkpoint inhibitors. Published literature was reviewed using available databases (PubMed, Cochrane, Scopus) and web pages (clinicaltrials...
August 22, 2017: Expert Review of Hematology
https://www.readbyqxmd.com/read/28803491/optimal-long-term-pharmacological-treatment-of-patients-with-venous-thromboembolism-that-was-unprovoked-or-associated-with-weak-risk-factors
#12
Gualtiero Palareti
Unprovoked venous thromboembolism (VTE) tend to recur. Many factors, patient- or event-related, influence the individual risk of recurrence. After initial and long-term (usually for 3-6 months) anticoagulant therapy, extended anticoagulation has been recommended in patients at high risk of recurrence, provided they do not have a high risk of bleeding. Areas covered: The effect of different risk factors on the risk of recurrence is discussed, as well as risk factors for bleeding. The estimation of individual balance between these two risks is crucial to decide which can be the best treatment duration in single patients...
August 21, 2017: Expert Review of Hematology
https://www.readbyqxmd.com/read/28799432/targeted-therapies-in-acute-myeloid-leukemia-a-focus-on-flt-3-inhibitors-and-abt199
#13
Kiran Naqvi, Marina Konopleva, Farhad Ravandi
Acute myeloid leukemia (AML) remains a therapeutic challenge. Despite ongoing research, the standard therapy for AML has not changed significantly in the past four decades. With the identification of cytogenetic and molecular abnormalities, several promising therapeutic agents are currently being investigated. FLT3 mutation is a well-recognized target seen in 30% of the cytogenetically normal AML. More recently, the BCL2 family of anti-apoptotic proteins have also generated great interest as a therapeutic target...
August 21, 2017: Expert Review of Hematology
https://www.readbyqxmd.com/read/28756726/mogamulizumab-for-the-treatment-of-relapsed-or-refractory-adult-t-cell-leukemia-lymphoma
#14
Frank T Winsett, Daniel J Lewis, Madeleine Duvic
Adult T-cell leukemia-lymphoma (ATL) is an aggressive variant of peripheral T-cell lymphoma of CD4+ T-malignant cells caused by human T-lymphotropic virus type-1. Despite aggressive treatment with multidrug combination chemotherapies, ATL confers a poor prognosis and commonly develops resistance to conventional treatments. Areas covered: Mogamulizumab is a humanized, defucosylated monoclonal antibody that acts by targeting the CC chemokine receptor 4 (CCR4) on malignant cells of ATL. In phase I and II clinical trials, it has achieved overall response rates of 31-50% in CCR4+ malignancies...
August 21, 2017: Expert Review of Hematology
https://www.readbyqxmd.com/read/28806107/the-case-for-lymphoma-specific-survival
#15
Marc Sorigue, Edurne Sarrate, Josep-Maria Ribera, Juan-Manuel Sancho
No abstract text is available yet for this article.
August 14, 2017: Expert Review of Hematology
https://www.readbyqxmd.com/read/28742419/insights-into-the-management-of-chronic-myeloid-leukemia-in-resource-poor-settings-a-mexican-perspective
#16
Andrés Gomez-de-León, David Gómez-Almaguer, Guillermo J Ruiz-Delgado, Guillermo J Ruiz-Arguelles
The arrival of targeted therapy for chronic myeloid leukemia (CML) was revolutionary. However, due to the high cost of tyrosine kinase inhibitors, access to this highly effective therapy with strict monitoring strategies is limited in low to middle-income countries. In this context, following standard recommendations proposed by experts in developed countries is difficult. Areas covered: This review aims to provide an insight into the management of patients with CML living in a resource-limited setting. It addresses several issues: diagnosis, initial treatment, disease monitoring, and additional treatment alternatives including allogeneic hematopoietic stem cell transplantation...
August 3, 2017: Expert Review of Hematology
https://www.readbyqxmd.com/read/28741391/clinical-implications-benefits-and-pitfalls-of-using-and-reversing-non-vitamin-k-antagonist-oral-anticoagulants
#17
Alexandra Sophie Moss, Gerasimos Dimitropoulous, Gregory Y H Lip
The use of non-Vitamin K antagonist oral anticoagulant (NOAC) drugs is increasingly common in clinical practice. As compared to vitamin K antagonists they are more straightforward to initiate, require no hematological monitoring and offer potentially more stable therapeutic indices. Concern has been raised with regard to their safety profiles particularly in the context of acute reversal in major bleeding. Further issues pertain to patient concordance. Areas covered: This review article aims to provide an overview of the current evidence relating to NOAC safety as well as the management of NOAC-related major bleeding with particular emphasis on reversal agents in use and in development following a selective literature review...
July 31, 2017: Expert Review of Hematology
https://www.readbyqxmd.com/read/28712322/is-now-the-time-for-molecular-driven-therapy-for-diffuse-large-b-cell-lymphoma
#18
Alice Di Rocco, Federico De Angelis, Michela Ansuinelli, Robin Foà, Maurizio Martelli
Recent genetic and molecular discoveries regarding alterations in diffuse large B-cell lymphoma (DLBCL) deeply changed the approach to this lymphoproliferative disorder. Novel additional predictors of outcomes and new therapeutic strategies are being introduced to improve outcomes. Areas covered: This review aims to analyse the recent molecular discoveries in DLBCL, the rationale of novel molecular driven treatments and their impact on DLBCL prognosis, especially in ABC-DLBCL and High Grade B Cell Lymphoma...
July 25, 2017: Expert Review of Hematology
https://www.readbyqxmd.com/read/28675317/emerging-applications-of-gene-edited-t-cells-for-the-treatment-of-leukemia
#19
Christos Georgiadis, Waseem Qasim
No abstract text is available yet for this article.
July 25, 2017: Expert Review of Hematology
https://www.readbyqxmd.com/read/28724345/simultaneous-bilateral-total-knee-arthroplasty-in-hemophilia-is-it-recommended
#20
E Carlos Rodriguez-Merchan
Simultaneous bilateral total knee arthroplasty (TKA) is a potentially cost-saving manner of caring for patients with hemophilia who have bilateral symptomatic hemophilic knee arthropathy. Because factor VIII is used only once in bilateral TKA, it is performed in hemophilia to reduce costs. Areas covered: This article reviews the potential role of simultaneous bilateral TKA in patients with hemophilia. Expert commentary: Although two recent reports have mentioned the benefits of simultaneous bilateral TKA in patients with hemophilia, the data are still scant and preliminary...
July 20, 2017: Expert Review of Hematology
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