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Expert Review of Hematology

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https://www.readbyqxmd.com/read/28632418/therapeutic-monitoring-of-unfractionated-heparin-trials-and-tribulations
#1
Israfil Baluwala, Emmanuel Favaloro, Leonardo Pasalic
Heparin is one of the oldest biological medicines with an established role in prevention and treatment of arterial and venous thromboembolism. Published therapeutic ranges for unfractionated heparin (UFH) mostly precede the large increase in the number of activated partial thromboplastin time (APTT) reagent/instrument combinations that now show wide variability. Areas covered: This paper explores the use of UFH, the development of heparin therapeutic ranges (HTRs), and the strengths and limitations of the methods used to monitor heparin's anticoagulant effect...
June 20, 2017: Expert Review of Hematology
https://www.readbyqxmd.com/read/28592170/current-options-to-manage-waldenstr%C3%A3-m-s-macroglobulinemia
#2
Giulia Benevolo, Maura Nicolosi, Elisa Santambrogio, Umberto Vitolo
Waldenström's macroglobulinemia (WM) is a rare, incurable B-cell lymphoma, with a median survival of 5-10 years in symptomatic patients. There is no consensus on the standard of care and several agents are currently used in these patients. Areas covered: In this article, we will review the use of standard therapies and new drugs investigated such as monoclonal antibodies, proteasome inhibitors, immunomodulatory agents, Bruton's tyrosine kinase inhibitors and novel agents in early-stage development. Expert commentary: RCD (Rituximab/Cyclophosphamide/Dexamethasone) is an effective and safe treatment in first line in WM...
June 19, 2017: Expert Review of Hematology
https://www.readbyqxmd.com/read/28586248/side-effects-and-potential-risk-factors-of-botulinum-toxin-type-a-intramuscular-injections-in-knee-flexion-contractures-of-hemophiliacs
#3
E Carlos Rodriguez-Merchan, Hortensia De la Corte-Rodriguez
Knee flexion contracture (KFC) is a common complication of recurrent hemarthrosis in children and young adults with hemophilia. If the KFC is not prevented (by means of primary prophylaxis) and treated properly and early (be means of physical medicine and rehabilitation), it will become fixed. Areas covered: The aim of this article is to review the potential role of botulinum toxin type A (BTX-A) intramuscular injections for the treatment of KFC in people with hemophilia (PWH). Expert commentary: Although two recent reports have mentioned the benefits of intramuscular injections of BTX-A in PWH with KFC, the data are still scant and too preliminary...
June 19, 2017: Expert Review of Hematology
https://www.readbyqxmd.com/read/28571503/managing-side-effects-of-jak-inhibitors-for-myelofibrosis-in-clinical-practice
#4
Iram Saeed, Donal McLornan, Claire N Harrison
Myelofibrosis (MF) is characterized by bone marrow fibrosis, abnormalities in peripheral counts, extramedullary hematopoiesis, splenomegaly and an increased risk of transformation to acute myeloid leukaemia. The disease course is often heterogeneous and management can range from observation alone through to allogeneic stem cell transplantation. As of 2017, the only approved medication for MF remains the JAK Inhibitor (JAKi), ruxolitinib (Novartis Pharmaceuticals, Basel, Switzerland; Incyte, Wilmington, Detroit, USA) although several others have reached advanced stages of clinical trials...
June 19, 2017: Expert Review of Hematology
https://www.readbyqxmd.com/read/28617144/the-risk-of-stroke-among-acutely-ill-hospitalized-medical-patients-lessons-from-recent-trials-on-extended-duration-thromboprophylaxis
#5
Jolanta Marszalek, Sara Mehrsefat, Gerald Chi
Data from recent randomized controlled trials indicate that the incidence of stroke among acutely ill medical patients is unexpectedly high and approximates 1% at 90 days. Preliminary data suggest that betrixaban may reduce ischemic stroke in patients without atrial fibrillation. There is an unmet demand for stroke risk stratification schemes targeting hospitalized medical patients. The prognostic value of biomarkers such as natriuretic peptides and D-dimer in predicting short-term stroke remains uncertain...
June 15, 2017: Expert Review of Hematology
https://www.readbyqxmd.com/read/28617062/novel-approaches-to-targeting-myd88-in-waldenstr%C3%A3-m-macroglobulinemia
#6
Jorge J Castillo, Zachary R Hunter, Guang Yang, Steven P Treon
Waldenström macroglobulinemia (WM) is an incurable lymphoma characterized by the accumulation of IgM-producing lymphoplasmacytic cells in the bone marrow and other organs. Although WM patients can experience prolonged remissions, the disease invariably recurs advocating for the need of novel treatments in order to achieve higher response and survival rates. The discovery of a recurrent mutation in the MYD88 gene and an increased understanding behind the biology of MYD88 signaling have provided the opportunity to developing novel agents targeting the MYD88 pathway...
June 15, 2017: Expert Review of Hematology
https://www.readbyqxmd.com/read/28598700/comment-and-response-to-the-significance-of-early-warning-in-chronic-myeloid-leukemia
#7
Ahmet Emre Eskazan, Muhlis Cem Ar, Teoman Soysal
No abstract text is available yet for this article.
June 15, 2017: Expert Review of Hematology
https://www.readbyqxmd.com/read/28598209/the-significance-of-early-warning-in-chronic-myeloid-leukemia
#8
Mario Tiribelli, Gianni Binotto, Massimiliano Bonifacio
No abstract text is available yet for this article.
June 15, 2017: Expert Review of Hematology
https://www.readbyqxmd.com/read/28586251/the-relationship-between-idiopathic-cytopenias-dysplasias-of-uncertain-significance-icus-idus-and-autoimmunity
#9
Wilma Barcellini
This review examines the several lines of evidence that support the relationship between myelodysplasia and autoimmunity, i.e. their epidemiologic association, the existence of common immune-mediated physiopathologic mechanisms, and the response to similar immunosuppressive therapies. The same relationship is reviewed here considering idiopathic cytopenia of uncertain significance (ICUS) and idiopathic dysplasia of uncertain significance (IDUS), two recently recognized provisional conditions characterized by isolated/unexplained cytopenia and/or dysplasia in <10% bone marrow cells...
June 15, 2017: Expert Review of Hematology
https://www.readbyqxmd.com/read/28586242/tyrosine-kinase-inhibitor-tki-therapy-for-newly-diagnosed-patients-with-chronic-myeloid-leukemia-focusing-on-tki-discontinuation-due-to-adverse-events-is-better-always-good
#10
Ahmet Emre Eskazan, Deniz Ozmen
No abstract text is available yet for this article.
June 13, 2017: Expert Review of Hematology
https://www.readbyqxmd.com/read/28503958/neonatal-management-and-outcome-in-alloimmune-hemolytic-disease
#11
Isabelle M C Ree, Vivianne E H J Smits-Wintjens, Johanna G van der Bom, Jeanine M M van Klink, Dick Oepkes, Enrico Lopriore
Hemolytic disease of the fetus and newborn (HDFN) occurs when fetal and neonatal erythroid cells are destroyed by maternal erythrocyte alloantibodies, it leads to anemia and hydrops in the fetus, and hyperbilirubinemia and kernicterus in the newborn. Postnatal care consists of intensive phototherapy and exchange transfusions to treat severe hyperbilirubinemia and top-up transfusions to treat early and late anemia. Other postnatal complications have been reported such as thrombocytopenia, iron overload and cholestasis requiring specific management...
June 5, 2017: Expert Review of Hematology
https://www.readbyqxmd.com/read/28504024/detection-of-driver-and-subclonal-mutations-in-myelofibrosis-clinical-impact-on-pharmacologic-and-transplant-based-treatment-strategies
#12
Maria Chiara Finazzi, Federico Lussana, Silvia Salmoiraghi, Orietta Spinelli, Alessandro Rambaldi
Myelofibrosis (MF) is the most aggressive form among Philadelphia negative (Ph-) myeloproliferative neoplasms (MPNs). In the last years, the mutational landscape of MF has expanded remarkably by the identification of additional recurrent mutations, called subclonal mutations. Areas covered: Here we describe the available data about the currently identified subclonal mutations and their prognostic value in MF patients. We also review the practical value of including such molecular information in available prognostic models for both outcome prediction and possibly treatment decision with regards to transplant indication...
May 25, 2017: Expert Review of Hematology
https://www.readbyqxmd.com/read/28511567/treating-the-chronic-phase-chronic-myeloid-leukemia-patient-which-tki-when-to-switch-and-when-to-stop
#13
Ami B Patel, Brandon W Wilds, Michael W Deininger
With the discovery of imatinib mesylate nearly 20 years ago, tyrosine kinase inhibitors (TKIs) were found to be effective in chronic myeloid leukemia (CML). TKI therapy has since revolutionized the treatment of CML and has served as a paradigm of success for targeted drug therapy in cancer. Several new TKIs for CML have been approved over the last two decades that exhibit improved potency over imatinib and have different off-target profiles, providing options for individualized therapy selection. Areas covered: Current management of chronic phase CML, including guidance on the sequential use of imatinib and newer-generation TKIs and evolving treatment strategies such as TKI discontinuation...
May 22, 2017: Expert Review of Hematology
https://www.readbyqxmd.com/read/28504554/how-is-patient-care-for-multiple-myeloma-advancing
#14
REVIEW
Sonja Genadieva Stavric, Francesca Bonello, Sara Bringhen, Mario Boccadoro, Alessandra Larocca
Treatment of multiple myeloma has undergone profound changes in the past years thanks to the increased understanding of the biology of the disease and the new treatment options. New drugs and effective approaches are currently available for the treatment of multiple myeloma, including immunomodulatory agents, proteasome inhibitors and autologous stem cell transplantation. Areas covered: We have described the recent updated criteria to start treatment in multiple myeloma and summarized clinical data from major studies including most recent agents...
June 2017: Expert Review of Hematology
https://www.readbyqxmd.com/read/28482712/heme-mediated-cell-activation-the-inflammatory-puzzle-of-sickle-cell-anemia
#15
REVIEW
Caroline Conceição da Guarda, Rayra Pereira Santiago, Luciana Magalhães Fiuza, Milena Magalhães Aleluia, Júnia Raquel Dutra Ferreira, Camylla Vilas Boas Figueiredo, Setondji Cocou Modeste Alexandre Yahouedehou, Rodrigo Mota de Oliveira, Isa Menezes Lyra, Marilda de Souza Gonçalves
Hemolysis triggers the onset of several clinical manifestations of sickle cell anemia (SCA). During hemolysis, heme, which is derived from hemoglobin (Hb), accumulates due to the inability of detoxification systems to scavenge sufficiently. Heme exerts multiple harmful effects, including leukocyte activation and migration, enhanced adhesion molecule expression by endothelial cells and the production of pro-oxidant molecules. Area covered: In this review, we describe the effects of heme on leukocytes and endothelial cells, as well as the features of vascular endothelial cells related to vaso-occlusion in SCA...
June 2017: Expert Review of Hematology
https://www.readbyqxmd.com/read/28480766/follicular-lymphomas-and-their-transformation-past-and-current-research
#16
REVIEW
Miriam Mendez, Maria Torrente, Mariano Provencio
Follicular lymphoma (FL) is the second most common type of non-Hodgkin lymphoma (NHL). Histological transformation (HT) refers to the evolution of a clinically indolent NHL to a clinically aggressive one, defined as those lymphomas in which survival is limited to a few months when untreated. Areas covered: HT is associated with rapid progression of lymphadenopathy, infiltration of extranodal sites, development of systemic symptoms, and elevated serum level of lactate dehydrogenase (LDH). It is frequently related to a poor prognosis, and the median survival after transformation is less than 2 years...
June 2017: Expert Review of Hematology
https://www.readbyqxmd.com/read/28475434/enhancing-acute-myeloid-leukemia-therapy-monitoring-response-using-residual-disease-testing-as-a-guide-to-therapeutic-decision-making
#17
REVIEW
Benjamin Tomlinson, Hillard M Lazarus
Current standards for monitoring the response of acute myeloid leukemia (AML) are based on morphologic assessments of the bone marrow and recovery of peripheral blood counts. A growing experience is being developed to enhance the detection of small amounts of AML, or minimal residual disease (MRD). Areas covered: Available techniques include multi-color flow cytometry (MFC) of leukemia associated immunophenotypes (LAIP), quantitative reverse transcriptase polymerase chain reaction (QRT-PCR) for detecting fusion and mutated genes (RUNX1-RUNX1T1, CBFB-MYH11, and NPM1), overexpression of genes such as WT1, and next generation sequencing (NGS) for MRD...
June 2017: Expert Review of Hematology
https://www.readbyqxmd.com/read/28471695/current-use-and-potential-role-of-procalcitonin-in-the-diagnostic-work-up-and-follow-up-of-febrile-neutropenia-in-hematological-patients
#18
REVIEW
Benedetto Bruno, Alessandro Busca, Stefano Vallero, Stefania Raviolo, Nicola Mordini, Luca Nassi, Alessandro Cignetti, Ernesta Audisio, Moreno Festuccia, Alessandra Corsetti, Lorella Depaoli, Maura Faraci, Concetta Micalizzi, Silvia Corcione, Massimo Berger, Francesco Saglio, Paola Caropreso, Giulio Mengozzi, Vincenzo Squadrone, Francesco Giuseppe De Rosa, Luisa Giaccone
Febrile neutropenia (FN) represents a life-threatening complication in hematological malignancies. Its etiology is most often due to infections even though FN of other origins, such as tumor-related fever and non-infectious inflammation, should rapidly be ruled out. Initially, C-reactive protein and, more recently, procalcitonin (PCT) have been proposed as useful biomarkers for differential diagnosis. PCT was shown to be a good biomarker of bacterial infections and their clinical outcomes. Definition of standard cut-offs and design of PCT-guided treatment protocols remain however to be defined...
June 2017: Expert Review of Hematology
https://www.readbyqxmd.com/read/28468596/multiple-viral-infections-in-primary-effusion-lymphoma-a-model-of-viral-cooperation-in-lymphomagenesis
#19
REVIEW
Annunziata Gloghini, Chiara C Volpi, Ambra V Gualeni, Riccardo Dolcetti, Italia Bongarzone, Paolo De Paoli, Antonino Carbone
Primary effusion lymphoma (PEL) is a rare B-cell lymphoid neoplasm mainly associated with HIV infection, presenting as pleural, peritoneal, and pericardial effusions. A defining property of PEL is its consistent association with Kaposi sarcoma associated herpesvirus (KSHV) infection, and, in most cases, Epstein Barr virus (EBV) co-infection. On these grounds, a review of the literature related to viral cooperation and lymphomagenesis can help to understand the complex interplay between KSHV and EBV in PEL pathogenesis...
June 2017: Expert Review of Hematology
https://www.readbyqxmd.com/read/28460542/hematological-disorders-at-birth-in-complicated-monochorionic-twins
#20
REVIEW
Lianne Verbeek, Femke Slaghekke, Marieke Sueters, Johanna M Middeldorp, Frans J Klumper, Monique C Haak, Dick Oepkes, Enrico Lopriore
Monochorionic twins are at risk of severe complications including twin-twin transfusion syndrome (TTTS), twin anemia-polycythemia sequence (TAPS) and acute peripartum TTTS. The pathophysiology is based on inter-twin blood transfusion through placental vascular anastomoses. Areas covered: This review focuses on the incidence, management and outcome of neonatal hematological complications at birth in TTTS, TAPS and acute peripartum TTTS. Expert commentary: Hematological disorders are often present at birth in monochorionic twins and include acute or chronic anemia, polycythemia and thrombocytopenia...
June 2017: Expert Review of Hematology
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