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Expert Review of Hematology

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https://www.readbyqxmd.com/read/28395545/lymphoma-classification-update-b-cell-non-hodgkin-lymphomas
#1
Manli Jiang, N Nora Bennani, Andrew L Feldman
Lymphomas are classified based on the normal counterpart, or cell of origin, from which they arise. Because lymphocytes have physiologic immune functions that vary both by lineage and by stage of differentiation, the classification of lymphomas arising from these normal lymphoid populations is complex. Recent genomic data have contributed additional complexity. Areas covered: Lymphoma classification follows the World Health Organization (WHO) system, which reflects international consensus and is based on pathological, genetic, and clinical factors...
April 24, 2017: Expert Review of Hematology
https://www.readbyqxmd.com/read/28388244/which-therapies-will-move-to-the-front-line-for-multiple-myeloma
#2
María J Cejalvo, Javier de la Rubia
Despite substantial progress, multiple myeloma (MM) remains an incurable disease. Recently the availability of several novel drugs with different and innovative mechanisms of action (daratumumab, elotuzumab, carfilzomib, ixazomib, and panobinostat) has increased the therapeutic options but has also increased complexity in the management of patients with MM. Areas covered: The outstanding results observed in the relapsed setting with regimens including these new drugs has provided the investigators with several treatment options that are being tested also in patients with newly diagnosed MM...
April 24, 2017: Expert Review of Hematology
https://www.readbyqxmd.com/read/28374619/super-resolution-microscopy-in-the-diagnosis-of-platelet-granule-disorders
#3
Alex E Knight, Keith Gomez, Daniel F Cutler
Platelet granule deficiencies lead to bleeding disorders, but their specific diagnosis typically requires whole mount transmission electron microscopy, which is often not available and has a number of important limitations. We recently proposed the use of advanced forms of fluorescence microscopy - the so-called 'super-resolution' microscopies - as a possible solution. Areas covered: In this special report, we review the diagnosis of platelet granule deficiencies, and discuss how recent developments in fluorescence microscopy may be useful in improving diagnostic approaches to these and related disorders...
April 13, 2017: Expert Review of Hematology
https://www.readbyqxmd.com/read/28402197/minimal-residual-disease-or-cure-in-mpns-rationales-and-perspectives-on-combination-therapy-with-interferon-alpha2-and-ruxolitinib
#4
Mads Emil Bjørn, Hans Carl Hasselbalch
The therapeutic landscape of the Philadelphia-negative myeloproliferative neoplasms (MPNs) is markedly changing consequent to the development of JAK-inhibitors and the use of ruxolitinib (RUX) in patients with myelofibrosis (MF) and patients with polycythemia vera (PV) who develop refractoriness or intolerance to hydroxyurea. The use of Interferon-alpha2 (IFN) is rapidly expanding in several countries, based upon favourable safety and efficacy profiles in several single-arm studies during the last 30 years, displaying complete hematological remissions in a large proportion of patients, a reduction in the JAK2V617 F and CALR mutational burden and in a subset of patients with PV with normalisation of the bone marrow after long-term treatment - even being sustained for several years after discontinuation of IFN...
April 12, 2017: Expert Review of Hematology
https://www.readbyqxmd.com/read/28402148/should-modulation-of-p50-be-a-therapeutic-target-in-the-critically-ill
#5
Amudan J Srinivasan, Clare Morkane, Daniel S Martin, Ian J Welsby
A defining feature of human hemoglobin is its oxygen binding affinity, quantified by the partial pressure of oxygen at which hemoglobin is 50% saturated (p50), and the variability of this parameter over a range of physiological and environmental states. Modulation of this property of hemoglobin can directly affect the degree of peripheral oxygen offloading and tissue oxygenation. Areas covered: This review summarizes the role of hemoglobin oxygen affinity in normal and abnormal physiology and discusses the current state of the literature regarding artificial modulation of p50...
April 12, 2017: Expert Review of Hematology
https://www.readbyqxmd.com/read/28359170/immunotherapy-for-the-treatment-of-hodgkin-lymphoma
#6
Eva M Donato, Miguel Fernández-Zarzoso, Javier De La Rubia
Most patients with Hodgkin lymphoma (HL) enjoy durable remissions following front-line treatment but 30% of patients are refractory or relapse after first line therapy. Salvage chemotherapy followed by autologous stem cell transplantation (ASCT) can cure an additional 50-55% of relapsing patients but new treatments are needed for patients with HL who are refractory or relapse after ASCT. Immunotherapy has emerged as a promising treatment for the management of these patients. The availability of the anti-CD30 antibody brentuximab vedotin and new targeted drugs such as immune checkpoint inhibitors, show promising clinical activity in patients with HL and are important milestones for the management of patients with HL particularly for those who have progressed after standard initial therapy and ASCT...
April 12, 2017: Expert Review of Hematology
https://www.readbyqxmd.com/read/28395559/management-of-myelofibrosis-jak-inhibition-and-beyond
#7
Maximilian Stahl, Amer M Zeidan
Myelofibrosis (MF) is characterized by bone marrow fibrosis with subsequent extramedullary hematopoiesis and abnormal cytokine expression leading to splenomegaly, constitutional symptoms and cytopenias. The discovery of the JAK2 V617F mutation in the majority of MF patients has been followed by significant progress in drug development for MF. Areas covered: In this article, we review advances in the understanding of the underlying disease biology, prognostic assessment and therapeutic modalities for MF. We provide clinical trial evidence behind using the JAK2 inhibitor ruxolitinib, erythropoiesis stimulating agents, androgens, immunomodulatory drugs, interferon, cytoreductive drugs and hypomethylating agents in MF...
April 11, 2017: Expert Review of Hematology
https://www.readbyqxmd.com/read/28395546/post-transplant-cyclophosphamide-a-promising-anti-graft-versus-host-disease-prophylaxis-where-do-we-stand
#8
Alberto Mussetti, Raffaella Greco, Jacopo Peccatori, Paolo Corradini
Post transplant cyclophosphamide (PT/Cy) in association to other immunosuppressive agents or alone has emerged as a promising pharmacological strategy in the setting of allogeneic hematopoietic cell transplant (allo-HCT). Its safety profile and effectiveness in reducing GvHD (acute GvHD incidence comprised between 15 and 30%, chronic GvHD 20-30% in the haploidentical setting) contributed to the spreading of this technique all over the world. Areas covered: This review summarizes the use of PT/Cy in the setting of allo-HCT, both for oncological and non-malignant hematological diseases...
April 11, 2017: Expert Review of Hematology
https://www.readbyqxmd.com/read/28349723/targeted-therapy-in-the-treatment-of-chronic-lymphocytic-leukemia-facts-shortcomings-and-hopes-for-the-future
#9
Stefano Molica
The therapy for chronic lymphocytic leukemia (CLL) is undergoing a major transformation. However, the seminal progresses realized to date with the use of novel agents, leave many practical questions unanswered. Areas covered: This review focuses on the recent data of the literature of small-kinase inhibitor (KI) molecules and how results of KI clinical trials may translate into current clinical practice. Several questions such as the advantage of combining small-KI molecules with anti-CD20 monoclonal antibodies or with chemo-immunotherapy in comparison to targeted agents alone are discussed...
March 28, 2017: Expert Review of Hematology
https://www.readbyqxmd.com/read/28290728/diffuse-large-b-cell-lymphoma-in-the-elderly-standard-treatment-and-new-perspectives
#10
Annalisa Chiappella, Alessia Castellino, Maura Nicolosi, Elisa Santambrogio, Umberto Vitolo
Diffuse large B-cell lymphoma (DLBCL) is the most common histotype in non Hodgkin lymphoma, with a peak incidence in the sixth decade. The standard treatment for elderly FIT DLBCL patients is Rituximab-CHOP; in unfit and frail patients, chemotherapy at reduced intensity should be considered. Areas covered: In this article, we will review use of standard therapies and new drugs investigated such as immonomudulating agents (IMiDs), Bruton Tyrosine Kinase (BTK), in fit, unfit, frail and very elderly DLCBL patients...
March 27, 2017: Expert Review of Hematology
https://www.readbyqxmd.com/read/28277805/intrauterine-transfusion-and-non-invasive-treatment-options-for-hemolytic-disease-of-the-fetus-and-newborn-review-on-current-management-and-outcome
#11
Carolien Zwiers, Inge van Kamp, Dick Oepkes, Enrico Lopriore
Hemolytic disease of the fetus and newborn (HDFN) remains a serious pregnancy complication which can lead to severe fetal anemia, hydrops and perinatal death. Areas covered: This review focusses on the current prenatal management, treatment with intrauterine transfusion (IUT) and promising non-invasive treatment options for HDFN. Expert commentary: IUTs are the cornerstone in prenatal management of HDFN and have significantly improved perinatal outcome in the past decades. IUT is now a relatively safe procedure, however the risk of complications is still high when performed early in the second trimester...
March 20, 2017: Expert Review of Hematology
https://www.readbyqxmd.com/read/28277849/targeting-indolent-non-hodgkin-lymphoma
#12
Lori A Leslie, Alan P Skarbnik, Coleen Bejot, Susan Stives, Tatyana A Feldman, Andre H Goy
Due to recent advancements in the understanding of the molecular pathogenesis of B-cell malignancies, there has been an explosion of innovative agents in development. The purpose of this review is to efficiently summarize novel therapies with activity in indolent non-Hodgkin lymphoma (iNHL) targeting surface antigens, signaling pathways, and the tumor microenvironment. Areas covered: A literature search was performed to identify preclinical data and clinical trials focused on the use of targeted therapies in iNHL subtypes including follicular lymphoma, marginal zone lymphoma, small lymphocytic lymphoma, and lymphoplasmacytic lymphoma/Waldenström macroglobulinemia...
March 15, 2017: Expert Review of Hematology
https://www.readbyqxmd.com/read/28277850/diagnosis-and-management-of-catastrophic-antiphospholipid-syndrome
#13
Or Carmi, Maya Berla, Yehuda Shoenfeld, Yair Levy
Catastrophic antiphospholipid syndrome (CAPS) is a rare, life-threatening disease. In 1992, Asherson defined it as a widespread coagulopathy related to the antiphospholipid antibodies (aPL). CAPS requires rapid diagnosis and prompt initiation of treatment. Areas covered: This paper discusses all aspects of CAPS, including its pathophysiology, clinical manifestations, diagnostic approaches, differential diagnoses, management and treatment of relapsing CAPS, and its prognosis. To obtain the information used in this review, scientific databases were searched using the key words antiphospholipid antibodies, catastrophic antiphospholipid syndrome, hemolytic anemia, lupus anticoagulant, and thrombotic microangiopathic hemolytic anemia...
March 13, 2017: Expert Review of Hematology
https://www.readbyqxmd.com/read/28264622/why-is-the-incidence-of-aplastic-anemia-higher-in-asia
#14
Seiji Kojima
No abstract text is available yet for this article.
March 13, 2017: Expert Review of Hematology
https://www.readbyqxmd.com/read/28277851/management-of-lower-risk-myelodysplastic-syndromes-without-del5q-current-approach-and-future-trends
#15
Maximilian Stahl, Amer M Zeidan
Myelodysplastic syndromes (MDS) are characterized by progressive bone marrow failure manifesting as blood cytopenia and a variable risk of progression into acute myeloid leukemia. MDS is heterogeneous in biology and clinical behavior. MDS is generally divided into lower-risk (LR) or higher-risk (HR) MDS. Goals of care in HR-MDS is changing the natural history of the disease, whereas in LR-MDS it is symptom control and quality of life. Areas covered: We review the epidemiology, tools of risk assessment, and the available therapeutic modalities for LR-MDS...
February 21, 2017: Expert Review of Hematology
https://www.readbyqxmd.com/read/28162019/interleukin-7-signaling-as-a-therapeutic-target-in-acute-lymphoblastic-leukemia
#16
Angela Maria Savino, Shai Izraeli
No abstract text is available yet for this article.
February 6, 2017: Expert Review of Hematology
https://www.readbyqxmd.com/read/28164722/when-to-treat-patients-with-relapsed-follicular-lymphoma
#17
Chan Yoon Cheah, John F Seymour
Follicular lymphoma is the most common indolent lymphoma and remains incurable for the majority of patients despite recent major advances. The disease is typically initially chemosensitive, however relapse is inevitable. In contrast to the frontline setting, studies addressing the optimal timing of initiating second line therapy have not been performed and subsequently practice varies considerably. Areas covered: In the review, we consider the available literature regarding timing of therapy in patients with follicular lymphoma and consider key insights from FL biology to provide guidance on when to treat patients with relapsed disease...
February 4, 2017: Expert Review of Hematology
https://www.readbyqxmd.com/read/28076695/deacetylase-inhibitors-an-advance-in-myeloma-therapy
#18
Jacob P Laubach, Jesus F San-Miguel, Vania Hungria, Jian Hou, Philippe Moreau, Sagar Lonial, Jae Hoon Lee, Hermann Einsele, Melissa Alsina, Paul G Richardson
A significant unmet need exists in patients with relapsed or refractory multiple myeloma (MM), which remains an incurable disease despite recent advances in the field. One such development was the use of deacetylase inhibitors (DACi), which exert unique antimyeloma effects through targeting of epigenetic and protein metabolism pathways. The pan-DACi panobinostat was recently approved in combination with bortezomib and dexamethasone for use in patients with relapsed or relapsed and refractory MM. Results of a phase 3 trial showed that the panobinostat-containing regimen improved the overall response rate and progression-free survival...
February 1, 2017: Expert Review of Hematology
https://www.readbyqxmd.com/read/28136133/the-gastrointestinal-tract-properties-and-role-in-allogeneic-hematopoietic-stem-cell-transplantation
#19
Ilias Pessach, Panagiotis D Tsirigotis, Arnon Nagler
The GI-tract is a major target for both the intensive chemo and/or radiotherapy conditioning as well as for GVHD and therefore is closely associated with transplant outcome. Apart from being a target, the GI-tract is also a mediator and therefore is also a key player of the pathogenetic process following allogeneic transplantation. Areas covered: The intestinal homeostasis is regulated through complicated interactions between the key players of this process which are the intestinal epithelium, the intestinal immune system, and the intestinal microbiota...
January 30, 2017: Expert Review of Hematology
https://www.readbyqxmd.com/read/28092987/emerging-treatment-approaches-for-myeloma-related-bone-disease
#20
Maria Gavriatopoulou, Meletios A Dimopoulos, Efstathios Kastritis, Evangelos Terpos
Multiple myeloma is characterized by the presence of osteolytic lesions that leads to devastating skeletal-related events in the majority of patients. Myeloma bone disease is attributed to increased osteoclastic and suppressed osteoblastic activity. Areas covered: Bisphosphonates remain the main treatment option, however they have limitations on their own. Understanding the pathogenesis of myeloma bone disease may provide a roadmap for new therapeutic approaches. The pathway of RANKRANKLOPG pathway has revealed denosumab, a monoclonal antibody targeting RANKL as a novel emerging therapy for myeloma-related bone disease...
January 29, 2017: Expert Review of Hematology
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