Current clinical strategies and emergent treatment landscapes in leukemic transformation of Philadelphia-negative myeloproliferative neoplasms.
Authors: Abruzzese E, Niscola P Abstract INTRODUCTION: Transformation to acute myeloid leukemia (AML) of Philadelphia chromosome-negative (Ph-) chronic myeloproliferative neoplasms (MPN) represents a challenging medical concern and an unmet clinical need, since it charts a very poor outcome and a low rate of response to standard treatments with the exception of allogeneic hematopoietic stem cell transplantation (HSCT). Recent novel insights into the molecular disease pathways and the genomic features characterizing the transformation of Ph-MPN have led to new therapeutic individualized approaches with the potential...
Source: Expert Review of Hematology - November 25, 2020 Category: Hematology Tags: Expert Rev Hematol Source Type: research

Apoptosis targeted therapies in acute myeloid leukemia: an update.
Authors: Ball S, Borthakur G Abstract INTRODUCTION: Management of acute myeloid leukemia (AML) continues to be a therapeutic challenge despite significant recent advancements. Dysregulation of several components of apoptotic pathways has been identified as potential driver in AML. AREAS COVERED: Overexpression of anti-apoptotic proteins, B-cell lymphoma 2 (BCL2), Bcl-xL, and myeloid cell leukemia-1 (MCL1), has been associated with worse outcome in AML. Dysfunction of p53 pathway (often through mouse double minute 2 homolog (MDM2)) and high expression of inhibitor of apoptosis proteins (IAP) constitute other dis...
Source: Expert Review of Hematology - November 21, 2020 Category: Hematology Tags: Expert Rev Hematol Source Type: research

Tyrosine kinase inhibitor discontinuation in the management of chronic myeloid leukemia: a critical review of the current practice.
Authors: Breccia M, Efficace F, Colafigli G, Scalzulli E, Di Prima A, Martelli M, Foà R Abstract Introduction: Tyrosine kinase inhibitors (TKIs), which target BCR-ABL1 kinase activity, have significantly prolonged the overall survival of patients affected by chronic myeloid leukemia (CML) and changed drastically the outcome. Evidences from several studies suggest that in patients who have achieved a sustained, stable and deep molecular response, TKI treatment can be safely discontinued with a close subsequent monitoring. Thus, a stable deep molecular response (DMR) has become a feasible treatment goal in CML...
Source: Expert Review of Hematology - November 21, 2020 Category: Hematology Tags: Expert Rev Hematol Source Type: research

An analysis of genetic targets for guiding clinical management of follicular lymphoma.
Authors: Alonso-Alonso R, Rodriguez M, Morillo D, Cordoba R, Piris MA Abstract Introduction: Follicular lymphoma (FL) is one of the most common non-Hodgkin lymphoma (NHL) types, where genomic studies have accumulated potentially useful information about frequently mutated genes and deregulated pathways, which has allowed to a better understanding of the molecular pathogenesis of this tumor and the complex interrelationship between the tumoral cells and the stroma. Areas covered: The results of the molecular studies performed on Follicular Lymphoma have been here reviewed, summarizing the results of the clinical tri...
Source: Expert Review of Hematology - November 14, 2020 Category: Hematology Tags: Expert Rev Hematol Source Type: research

Chemokine receptor CXCR4: An important player affecting the molecular-targeted drugs commonly used in hematological malignancies.
Authors: Li L, Chai Y, Wu C, Zhao L Abstract INTRODUCTION: A variety of molecular-targeted drugs have been widely used in hematological malignancies and have shown great advances. Nevertheless, as the use of drugs in clinical practice increases, the problem of relapse or of the disease being refractory to treatment is becoming apparent. This problem is closely related to the C-X-C chemokine receptor 4 (CXCR4). AREAS COVERED: This review focuses mainly on the effect of CXCR4 on molecular-targeted drug resistance in hematological malignancies as well as the clinical efficacy of CXCR4 antagonists combined with mol...
Source: Expert Review of Hematology - November 12, 2020 Category: Hematology Tags: Expert Rev Hematol Source Type: research

Novel and combination therapies for polycythemia vera and essential thrombocythemia: the dawn of a new era.
Authors: Bewersdorf JP, Zeidan AM Abstract INTRODUCTION: Essential thrombocythemia (ET) and polycythemia vera (PV) belong to the BCR-ABL1-negative myeloproliferative neoplasms and are characterized by the clonal proliferation of hematopoietic stem and progenitor cells. The contribution of aberrant immune regulation within the bone marrow microenvironment to ET and PV pathogenesis as well as the underlying molecular landscape is becoming increasingly understood. AREAS COVERED: Authors searched PubMed and conference abstracts in August 2020 for preclinical and clinical studies to provide an overview of the immune...
Source: Expert Review of Hematology - October 22, 2020 Category: Hematology Tags: Expert Rev Hematol Source Type: research

Advances in maintenance strategy in newly diagnosed multiple myeloma patients eligible for autologous transplantation.
Authors: Wahab A, Rafae A, Faisal MS, Mushtaq K, Ehsan H, Khakwani M, Ashraf A, Rehan T, Ahmed Z, Shah Z, Khan A, Anwer F Abstract INTRODUCTION: Multiple myeloma (MM) lacks curative therapy. Therefore, researchers continue to conduct studies in an effort to improve progression-free survival (PFS) and overall survival (OS). Maintenance therapy (MT) after autologous stem cell transplant (ASCT) was extensively studied in the last decade and now considered a standard approach. AREAS COVERED: This review evaluated the evidence and updates on various maintenance agents in newly diagnosed multiple myeloma (NDMM) after...
Source: Expert Review of Hematology - October 22, 2020 Category: Hematology Tags: Expert Rev Hematol Source Type: research

PTPN22 gene polymorphism as a genetic risk factor for primary immune thrombocytopenia in Egyptian children.
Authors: Hesham M, Hassan T, Fawzy A, Mohamed N, Alhejny E, Fathy M, El Gebaly S, Zakaria M Abstract OBJECTIVES: Several genetic and non-genetic risk factors are implicated in the etiology and pathogenesis of primary immune thrombocytopenia (ITP). Protein tyrosine phosphatase non-receptor 22 gene (PTPN22) plays an important role in regulation of signal transduction through the T-cell receptors. PTPN22 1858 C>T single nucleotide polymorphism was reported to be associated with increased risk of autoimmune diseases. There are very few studies investigating the role of PTPN22(SNP) 1858C > T in child...
Source: Expert Review of Hematology - October 20, 2020 Category: Hematology Tags: Expert Rev Hematol Source Type: research

Revisiting the treatment of anemia in the setting of chronic kidney disease, hematologic malignancies and cancer: perspectives with opinion and commentary.
Authors: Musio F Abstract INTRODUCTION: Anemia has and will continue to be a central theme in medicine particularly as clinicians are treating a burgeoning population of complex multi-organ system processes. As a result of multiple randomized controlled trials (RCTs), meta-analyses, and societal recommendations overly restrictive paradigms and under-administration of erythropoiesis stimulating agents (ESAs) have likely been followed by clinicians among all specialties. AREAS COVERED: A review of anemia in the context of chronic kidney disease, hematologic malignancies and cancer is presented with focus on the e...
Source: Expert Review of Hematology - October 10, 2020 Category: Hematology Tags: Expert Rev Hematol Source Type: research

What is the future of patient reported outcomes in sickle cell disease?
We describe the most highly recommended generic and disease-specific PRO tools in SCD and discuss the challenges of incorporating them in clinical practice. EXPERT OPINION: PRO measures are essential to incorporate into SCD clinical trials either as primary or secondary outcomes. The use of PRO measures in SCD facilitates a patient-centered approach, which is likely to lead to improved outcomes. Significant challenges remain in adapting PRO tools to routine clinical use and in developing countries. PMID: 33034214 [PubMed - as supplied by publisher] (Source: Expert Review of Hematology)
Source: Expert Review of Hematology - October 10, 2020 Category: Hematology Tags: Expert Rev Hematol Source Type: research

Developing strategies to reduce the duration of therapy for patients with myeloproliferative neoplasms.
Authors: Bar-Natan M, Hoffman R Abstract INTRODUCTION: All current treatment strategies for myeloproliferative neoplasms (MPN) patients with the exception of allogeneic stem cell transplant (ASCT) are continuously administered. Treatment approaches that reduce the degree of minimal residual disease (MRD) might permit possible drug holidays or potential cures. AREA COVERED: Authors discuss the presently available agents and those that are under clinical development that might induce a state of MRD and can be administered intermittently. Data extracted from a comprehensive search of peer review literature perform...
Source: Expert Review of Hematology - October 7, 2020 Category: Hematology Tags: Expert Rev Hematol Source Type: research

Bispecific antibodies in acute lymphoblastic leukemia therapy.
Authors: Guranda C, Anna L, Marcus L, Claudia BD, Monika B Abstract INTRODUCTION: Blinatumomab, first in a class of bispecific T-cell engagers, revolutionized treatment paradigm of B-cell precursor relapsed/refractory or minimal residual disease positive acute lymphoblastic leukemia (ALL) in adults and children, inducing deep remissions in a proportion of patients. However, significant numbers of patients do not respond or eventually relapse. Strategies for improvement of treatment outcomes are required. AREAS COVERED: This review discusses the main structural and functional features of blinatumomab, and its pl...
Source: Expert Review of Hematology - October 3, 2020 Category: Hematology Tags: Expert Rev Hematol Source Type: research

Reducing infectious complications after allogeneic stem cell transplant.
Authors: Bacigalupo A, Metafuni E, Amato V, Marquez Algaba E, Pagano L Abstract INTRODUCTION: Infections remain a significant problem, in patients undergoing an allogeneic hematopoietic stem cell transplant (HSCT) and efforts have been made over the years, to reduce the incidence, morbidity and mortality of infectious complications . AREAS COVERED: This manuscript is focused on the epidemiology, risk factors and prevention of infections after allogeneic HSCT. A systematic literature review was performed using the PubMed database, between November 2019 and January 2020, with the following MeSH terms: stem cell t...
Source: Expert Review of Hematology - October 1, 2020 Category: Hematology Tags: Expert Rev Hematol Source Type: research

D-dimer level in COVID-19 infection: a systematic review.
Authors: Rostami M, Mansouritorghabeh H Abstract INTRODUCTION: COVID-19 disease has spread worldwide from December 2019 to the present day; the early stage of this disease can be associated with high D-dimer, prolonged PT, and elevated levels of fibrinogen, indicating activation of coagulation pathways and thrombosis. In this article, we analyze the levels of D-dimer in patients with COVID-19. AREA COVERED: In the current study, three databases, PubMed, Scopus, Web of Science, searched using related keywords and information extracted from articles such as location, sample size, gender, age, coagulation test val...
Source: Expert Review of Hematology - October 1, 2020 Category: Hematology Tags: Expert Rev Hematol Source Type: research

Glucose-regulated protein 78 (GRP78) as a potential novel biomarker and therapeutic target in multiple myeloma.
Authors: Ninkovic S, Harrison SJ, Quach H Abstract INTRODUCTION: Glucose-regulated protein 78 (GRP78) is a stress-inducible molecular chaperone expressed within the endoplasmic reticulum where it acts a master regulator of the unfolded protein response (UPR) pathway. At times of ER stress, activation of the UPR, a multimolecular pathway, limits proteotoxicity induced by misfolded proteins. In malignancies, including multiple myeloma which is characterised by an excessive accumulation of misfolded immunoglobulins, GRP78 expression is increased, with notable translocation of GRP78 to the cell surface. Studies suggest...
Source: Expert Review of Hematology - September 30, 2020 Category: Hematology Tags: Expert Rev Hematol Source Type: research

An evaluation of ticagrelor for the treatment of sickle cell anemia.
Authors: Ribeiro-Filho J, Yahouédéhou SCMA, Pitanga TN, Santana SS, Adorno EV, Barbosa CG, Ferreira JRD, Pina ETG, Dos Santos Neres JS, Leite IPR, Lyra IM, Goncalves MS Abstract INTRODUCTION: Ticagrelor is an antiplatelet agent approved for the treatment of patients with an acute coronary syndrome or a history of myocardial infarction. Considering the evidence demonstrating that ticagrelor-mediated inhibition of platelet activation and aggregation have beneficial effects in the treatment of thrombotic conditions, clinical studies have been conducted to evaluate the use of this drug for the treatment o...
Source: Expert Review of Hematology - September 27, 2020 Category: Hematology Tags: Expert Rev Hematol Source Type: research

Electromagnetic radiation: a new charming actor in hematopoiesis?
Authors: Darvishi M, Mashati P, Kandala S, Paridar M, Takhviji V, Ebrahimi H, Zibara K, Khosravi A Abstract INTRODUCTION: Electromagnetic waves play indispensable roles in life. With technology improvements, the biologic effects of waves are quite imperative. Many studies addressed the outcomes of Electromagnetic field (EMF) on various biological functions such as cell proliferation, gene expression, epigenetic alterations, genotoxic, and carcinogenic effects, and its therapeutic applications in medicine. The impact of EMF on bone marrow (BM) is of high importance, however, EMF effects on BM hematopoiesis is not we...
Source: Expert Review of Hematology - September 23, 2020 Category: Hematology Tags: Expert Rev Hematol Source Type: research

COVID-19 in benign hematology: emerging challenges and special considerations for healthcare professionals.
Authors: Noun P, Ibrahim A, Hodroj MH, Bou-Fakhredin R, Taher AT Abstract INTRODUCTION: Many patients with inherited or acquired benign hematological disorders are at increased risk of developing severe complications from COVID-19. These patients, therefore, require specific advice regarding isolation and changes to their usual treatment schedules. Their disease can also be associated with significant burden, and they necessitate life-long and regular access to therapy, and regular follow-up consultations and hospital visits. The current COVID-19 pandemic is therefore presenting many challenges for these patients, ...
Source: Expert Review of Hematology - September 17, 2020 Category: Hematology Tags: Expert Rev Hematol Source Type: research

Treatment of hairy cell leukemia.
Authors: Chihara D, Kreitman RJ Abstract INTRODUCTION: Purine analogs made dramatic improvements for patients with hairy cell leukemia (HCL), but patients often relapse, require multiple treatments, and may become refractory. Major developments in treatment of relapsed/refractory HCL occurred with discovery of disease biology. New agents increase the complexity of clinical decision-making. AREAS COVERED: Anti-CD22 recombinant immunotoxin Moxetumomab Pasudotox (Moxe), CD20 Mabs rituximab and obinutuzumab, BRAF/MEK inhibitors vemurafenib and dabrafenib-trametinib, and Bruton's tyrosine kinase (BTK) inhibitor ibru...
Source: Expert Review of Hematology - September 9, 2020 Category: Hematology Tags: Expert Rev Hematol Source Type: research

Copy number variations in adult patients with chronic immune thrombocytopenia.
CONCLUSION: Identified CNV regions harbor several candidate genes, the functions of which might shed light on the pathogenesis of chronic ITP. PMID: 32885695 [PubMed - as supplied by publisher] (Source: Expert Review of Hematology)
Source: Expert Review of Hematology - September 6, 2020 Category: Hematology Tags: Expert Rev Hematol Source Type: research

Co-existence of mutations in myeloproliferative neoplasms and their clinical significance: a prognostic approach.
CONCLUSION: It seems that monitoring the status of mutations in MPNs and recognizing the co-existence of mutations (especially dual mutations) in order to determine prognosis and possibility of progression to acute form of leukemia can lead to the prediction of prognosis in MPN patients as well as establishment of better and more reliable therapeutic strategies for patients. PMID: 32886563 [PubMed - as supplied by publisher] (Source: Expert Review of Hematology)
Source: Expert Review of Hematology - September 6, 2020 Category: Hematology Tags: Expert Rev Hematol Source Type: research

The advantages and risks of ruxolitinib for the treatment of polycythemia vera.
Authors: Colafigli G, Scalzulli E, Pepe S, Di Prima A, Efficace F, Martelli M, Foà R, Breccia M Abstract INTRODUCTION: Polycythemia vera is a myeloproliferative neoplasm characterized by an increased red blood cells mass, risk of thromboembolic events and of transformation into secondary myelofibrosis and acute leukemia. The goal of treatment is to reduce the risk of fatal cardiovascular events reducing the hematocrit level with phlebotomies and low-dose aspirin. In high-risk patients (age>60 years or previous thromboembolic events) cytoreductive therapy is indicated. In this setting, resistance and/or in...
Source: Expert Review of Hematology - September 4, 2020 Category: Hematology Tags: Expert Rev Hematol Source Type: research

Management of higher risk myelodysplastic syndromes after hypomethylating agents failure: are we about to exit the black hole?
Authors: Bewersdorf JP, Zeidan AM Abstract INTRODUCTION: Hypomethylating agents (HMA) remain the mainstay of treatment for patients with higher-risk myelodysplastic syndromes (HR-MDS). However, complete responses to HMAs are seen in
Source: Expert Review of Hematology - September 4, 2020 Category: Hematology Tags: Expert Rev Hematol Source Type: research

A critical evaluation of caplacizumab for the treatment of acquired thrombotic thrombocytopenic purpura.
Authors: Gómez-Seguí I, Fernández-Zarzoso M, de la Rubia J Abstract INTRODUCTION: Acquired thrombotic thrombocytopenic purpura (aTTP) is a thrombotic microangiopathy caused by inhibitory autoantibodies against ADAMTS13 protein. Until recently, the combination of plasma exchange (PEX) and immunosuppression has been the standard front-line treatment in this disorder. However, aTTP-related mortality, refractoriness, and relapse are still a matter of concern. AREAS COVERED: The better understanding of the pathophysiological mechanisms of aTTP has allowed substantial improvements in the diagnosi...
Source: Expert Review of Hematology - September 4, 2020 Category: Hematology Tags: Expert Rev Hematol Source Type: research

Precision medicine in acute myeloid leukemia: where are we now and what does the future hold?
Authors: Megías-Vericat JE, Martínez-Cuadrón D, Solana-Altabella A, Montesinos P Abstract INTRODUCTION: Precision medicine has revolutionized the diagnostic and therapeutic management of acute myeloid leukemia (AML), from standardized schemes based on chemotherapy to tailored approaches according to molecular and genetic profile and targeted therapy. AREAS COVERED: The main topics of precision medicine in AML were reviewed in MEDLINE, EMBASE and Cochrane Central Register databases, and future directions in this therapeutic area were addressed. This review included targeted therapies, drug-s...
Source: Expert Review of Hematology - September 3, 2020 Category: Hematology Tags: Expert Rev Hematol Source Type: research

An evaluation of zanubrutinib, a BTK inhibitor, for the treatment chronic lymphocytic leukemia.
Authors: Geethakumari PR, Awan F Abstract INTRODUCTION: Recent years have seen tremendous increase in the availability of therapeutic options for the management of patients with chronic lymphocytic leukemia (CLL). Notable among those are Bruton's tyrosine kinase (BTK) and b-cell lymphoma-2 (bcl-2) inhibitors. AREAS COVERED: Here the authors provide a brief overview of the BTK signaling pathway as well as available, approved BTK inhibitors for CLL. In addition, they review pre-clinical and clinical data related to zanubrutinib and its use and CLL and other lymphoid malignancies. A comprehensive search of the Pub...
Source: Expert Review of Hematology - September 3, 2020 Category: Hematology Tags: Expert Rev Hematol Source Type: research

How to manage early-stage follicular lymphoma.
Authors: Cencini E, Fabbri A, Mecacci B, Bocchia M Abstract INTRODUCTION: early-stage follicular lymphoma (FL) is characterized by good prognosis and can be cured with involved-field radiotherapy (IF-RT) in most cases. PET scan is a milestone of diagnostic work-up, with the aim of identifying a truly-localized disease; however, staging in most of the studies was without PET. AREAS COVERED: we have searched in MEDLINE (inclusive dates 1994-2020) data about localized FL management. While high-quality evidence is lacking, current guidelines recommend IFRT or involved-site RT as first-line treatment in limited stag...
Source: Expert Review of Hematology - September 3, 2020 Category: Hematology Tags: Expert Rev Hematol Source Type: research

Resource limited centres can deliver treatment for children with acute lymphoblastic leukaemia with risk-stratified minimal residual diseasebased UKALL 2003 protocol with no modification with a good outcome.
CONCLUSION: It is feasible to follow and deliver chemotherapy as per the UKALL2003 protocol without any modifications in resource-limited setting.The survival rates have significantly improved over the years in our centre from 5 yr. EFSof 60% in 2010and now to3 year EFS of 85%. It is important to note that there was no treatment abandonment in our cohort. PMID: 32870048 [PubMed - as supplied by publisher] (Source: Expert Review of Hematology)
Source: Expert Review of Hematology - September 3, 2020 Category: Hematology Tags: Expert Rev Hematol Source Type: research

What COVID-19 can mean for people with hemophilia beyond the infection risk.
Authors: De la Corte-Rodriguez H, Alvarez-Roman MT, Rodriguez-Merchan EC, Jimenez-Yuste V Abstract INTRODUCTION: The coronavirus disease 2019 (COVID-19) pandemic represents an unprecedented global health crisis. To combat its effects, many governments have opted for strategies of social isolation that involve a radical change in people's behavior. AREAS COVERED: For patients with hemophilia, the negative consequences of these measures can be greater, given they modify aspects of health care and lifestyles needed to counteract the adverse effects of hemophilia. The long-term consequences of the pandemic on patie...
Source: Expert Review of Hematology - September 2, 2020 Category: Hematology Tags: Expert Rev Hematol Source Type: research

Patient-reported symptom burden of chronic graft versus host disease: a systematic literature review.
Authors: Csanádi M, Ágh T, Farkas-Ráduly S, Gros B, Tapprich C, Trudeau JJ, Lipton JH, Mattsson J Abstract INTRODUCTION: Chronic graft-versus-host disease (GVHD) is a life-threating complication of allogeneic hematopoietic stem cell transplantation (HSCT) leading to high morbidity and quality of life issues. We conducted a systematic literature review on the patient reported symptom burden of chronic GVHD. AREAS COVERED: English-language articles published between 2005 and November 2018 were searched using CENTRAL, EMBASE and MEDLINE. Studies that used the 2005 or 2015 National Institute of...
Source: Expert Review of Hematology - September 2, 2020 Category: Hematology Tags: Expert Rev Hematol Source Type: research

Recent real-world treatment patterns and outcomes in US patients with relapsed/refractory multiple myeloma.
CONCLUSIONS: Although a trend for improved rwPFS and rwOS with newer versus older treatments was suggested, additional treatment options to improve patient outcomes are needed. PMID: 32844683 [PubMed - as supplied by publisher] (Source: Expert Review of Hematology)
Source: Expert Review of Hematology - August 28, 2020 Category: Hematology Tags: Expert Rev Hematol Source Type: research

Moving on from 2013 to 2020 European LeukemiaNet recommendations for treating chronic myeloid leukemia: what has changed over the 7 years?
Authors: Yilmaz U, Eskazan AE PMID: 32814447 [PubMed - as supplied by publisher] (Source: Expert Review of Hematology)
Source: Expert Review of Hematology - August 23, 2020 Category: Hematology Tags: Expert Rev Hematol Source Type: research

Predicting the need for massive transfusion in the prehospital setting.
Authors: Puzio TJ, Kalkwarf K, Cotton BA Abstract INTRODUCTION: Massive transfusion (MT) prediction scores allowed for the early identification of patients with massive hemorrhage likely to require large volumes of blood products. Despite their utility, very few MT scoring systems have shown promise in the pre-hospital setting due to their complexity and resource limitations. AREAS COVERED: Pub med database was utilized to identify supporting literature for this review which discusses the importance of blood-based resuscitation and highlights the utility of scoring systems to predict the need of massive transfu...
Source: Expert Review of Hematology - August 13, 2020 Category: Hematology Tags: Expert Rev Hematol Source Type: research

Evolution of the role of haploidentical stem cell transplantation: past, present, and future.
Authors: Kwon M, Bailén R, Díez-Martín JL Abstract INTRODUCTION: The accessibility to haplo-donors has led to an increase in the number of haplo-HSCT worldwide. A systematic search of the PubMed database between 2000 to present was performed. AREAS COVERED: In this review, the authors discussed the most used approaches to perform haplo-HSCT and its results: T-cell depletion (TCD, including Perugia platform and its modifications) and T-cell repleted haplo (TCR, including the high-dose post-transplant cyclophosphamide strategy (Baltimore protocol) and the Beijing protocol). The improvements a...
Source: Expert Review of Hematology - August 13, 2020 Category: Hematology Tags: Expert Rev Hematol Source Type: research

Efficacy and safety of front-line treatments for advanced Hodgkin lymphoma: a systematic literature review.
CONCLUSION: New therapies, such as A+AVD, maintain the efficacy observed with current treatments, and may provide a more tolerable treatment option for patients with advanced-stage HL. PMID: 32749937 [PubMed - as supplied by publisher] (Source: Expert Review of Hematology)
Source: Expert Review of Hematology - August 13, 2020 Category: Hematology Tags: Expert Rev Hematol Source Type: research

Chemotherapy combinations for B-cell lymphoma and chemo-free approach in elderly patients: an update on best practice.
This article reviews the majority data in literature on immunochemotherapy regimens and chemo-free approaches available for DLBCL, FL, and MCL in the elderly, both in front-line and relapse/refractory setting, the incoming drugs and how to identify the best option for each patient. EXPERT OPINION: The therapeutic approach for elderly B-NHL is challenging and a tailored approach guided by a geriatric assessment is mandatory, in order to optimize efficacy and minimize treatment-related toxicities. The more extended use of biological drugs may potentially lead to prolonged survival with reduction of toxicities and improve...
Source: Expert Review of Hematology - August 4, 2020 Category: Hematology Tags: Expert Rev Hematol Source Type: research

Analysis of Hb levels and degree of anemia in relation to genotype in 615 patients with hemoglobin H disease.
CONCLUSION: Patients with Hb H disease in Guangxi have particular gene profiles. The clinical manifestations of various types of Hb H disease are heterogeneous; the Hb levels of patients with deletional Hb H are generally higher than those with non-deletional Hb H (P
Source: Expert Review of Hematology - August 1, 2020 Category: Hematology Tags: Expert Rev Hematol Source Type: research

Hypomethylating agent-based post-transplant strategies to maximize the outcome of high-risk acute myeloid leukemia after allogeneic stem cell transplantation.
Authors: Baek DW, Kim J, Cho HJ, Moon JH, Sohn SK Abstract INTRODUCTION: Clinical outcomes of patients diagnosed with high-risk acute myeloid leukemia (AML) are poor, and relapse or refractoriness is main cause of treatment failure, even in those who underwent standard allogeneic stem cell transplantation (allo-SCT). Therefore, innovative or additional approaches are necessary to overcome refractoriness to the graft-versus-leukemia (GVL) effect immediately after allo-SCT. AREAS COVERED: Hypomethylating agents (HMA) present a feasible option that can be adopted during the post-transplant phase. Moreover, combina...
Source: Expert Review of Hematology - August 1, 2020 Category: Hematology Tags: Expert Rev Hematol Source Type: research

Immunomodulatory drug- and proteasome inhibitor-backbone regimens in the treatment of relapsed multiple myeloma: an evidence-based review.
Authors: Sanchez L, Barley K, Richter J, Franz J, Cho HJ, Jagannath S, Madduri D, Parekh S, Richard S, Chari A Abstract INTRODUCTION: Recent advances and drug approvals in the last decade have substantially changed the landscape of relapsed and refractory multiple myeloma (RRMM), with not only improved outcomes for patients, but also increased complexity of treatment decisions. The approvals are based on randomized studies of novel agents added to an immunomodulatory drug (IMiD) or proteasome inhibitor (PI) backbone showing an improvement in outcomes. However, differences in enrolled patient populations/study desig...
Source: Expert Review of Hematology - August 1, 2020 Category: Hematology Tags: Expert Rev Hematol Source Type: research

Genetic risk factors for venous thromboembolism.
Authors: Zöller B, Svensson PJ, Dahlbäck B, Lind-Hallden C, Hallden C, Elf J Abstract INTRODUCTION: Venous thromboembolism (VTE) is a complex disease that aggregates in families. Both acquired and genetic risk factors are important. Proper recognition and management of high-risk individuals are important. AREAS COVERED: The genetic risk factors for VTE, the clinical consequences, and future perspectives are summarized. Classical thrombophilia i.e. factor V Leiden (rs6025), the prothrombin G20210A mutation (rs1799963), deficiencies of antithrombin, protein C, and protein S and the recent findings from ...
Source: Expert Review of Hematology - August 1, 2020 Category: Hematology Tags: Expert Rev Hematol Source Type: research

Novel challenges in the management of immunoglobulin light chain amyloidosis: from the bench to the bedside.
Authors: Basset M, Nuvolone M, Palladini G, Merlini G Abstract INTRODUCTION: Immunoglobulin light chain (AL) amyloidosis is one of the most frequent systemic amyloidosis in Western countries. It is caused by a B-cell clone producing a misfolded light chain (LC) that deposits in organs. AREAS COVERED: The review examines recent findings on pathophysiology and clinical management of AL amyloidosis. It contains an update on the recent hot topics as novel therapeutic approaches, definition of relapse and hematologic response assessment. To review literature on AL amyloidosis, a bibliographic search was performed us...
Source: Expert Review of Hematology - July 31, 2020 Category: Hematology Tags: Expert Rev Hematol Source Type: research

Complications of hemophilia in the elbow: current management.
Authors: Rodriguez-Merchan EC, De la Corte-Rodriguez H Abstract INTRODUCTION: After the ankle and knee, the elbow is the most commonly impaired joint in patients with hemophilia. Musculoskeletal problems in these patients begin in childhood, when small traumas cause repetitive hemarthroses in the elbow. AREAS COVERED: A Cochrane Library and PubMed (MEDLINE) search related to elbow problems in hemophilia was conducted. The main inclusion criterion was a focus on the musculoskeletal problems of hemophilia. Articles that did not focus on this topic were excluded. Early and continuous primary hematological prophyla...
Source: Expert Review of Hematology - July 31, 2020 Category: Hematology Tags: Expert Rev Hematol Source Type: research

An evaluation of polatuzumab vedotin for the treatment of patients with diffuse large B-cell lymphoma.
Authors: Walji M, Assouline S Abstract INTRODUCTION: Diffuse Large B-Cell lymphoma (DLBCL) is the most commonly diagnosed form of non-Hodgkin lymphoma (NHL) in adults. Most patients receive an initial treatment with chemo-immunotherapy, which includes rituximab, cyclophosphamide, vincristine, doxorubicin and prednisone (R-CHOP). Cure rates are high but those who relapse, or do not respond to initial therapy, have a poor prognosis. Polatuzumab vedotin, an anti-CD79b monoclonal antibody conjugated to the cytotoxic payload monomethyl aurostatin-E (MMAE), in combination with bendamustine and rituximab (polatuzumab-BR) ...
Source: Expert Review of Hematology - July 25, 2020 Category: Hematology Tags: Expert Rev Hematol Source Type: research

The combination of venetoclax and rituximab for the treatment of patients with recurrent chronic lymphocytic leukemia.
Authors: Vitale C, Ferrajoli A Abstract INTRODUCTION: In patients with recurrent chronic lymphocytic leukemia (CLL), treatment with targeted agents such as Bruton tyrosine kinase inhibitors and the Bcl-2 inhibitor venetoclax is rapidly replacing chemo-immunotherapy regimens. Venetoclax has demonstrated his efficacy as monotherapy in patients with previously treated CLL, and has been evaluated in combination with the anti-CD20 monoclonal antibody rituximab. AREAS COVERED: This review focuses on the activity and toxicity of the time-limited combination of venetoclax plus rituximab for the treatment of relapsed or...
Source: Expert Review of Hematology - July 25, 2020 Category: Hematology Tags: Expert Rev Hematol Source Type: research

An interview with Nancy Valente, the Global Head of Hematology Development at Roche.
Authors: Valente N PMID: 32684001 [PubMed - as supplied by publisher] (Source: Expert Review of Hematology)
Source: Expert Review of Hematology - July 22, 2020 Category: Hematology Tags: Expert Rev Hematol Source Type: research

Mechanisms of ibrutinib resistance in chronic lymphocytic leukemia and alternative treatment strategies.
Authors: Lama TG, Kyung D, O'Brien S Abstract INTRODUCTION: Development of the BTK inhibitor ibrutinib has changed the landscape of CLL treatment producing durable responses with minimal to no myelosuppression. Although remissions are durable, relapses remain a challenge. AREAS COVERED: Data from recent studies indicate that most patients relapsing on ibrutinib have mutations in BTK or PLCG2. The result of the C418S mutation in BTK is loss of covalent binding of ibrutinib to BTK resulting in reversible, transient inhibition in BTK mutant patients. There is downstream gain of function of BCR signaling with PLCG2...
Source: Expert Review of Hematology - July 18, 2020 Category: Hematology Tags: Expert Rev Hematol Source Type: research

Developments in identifying and managing mucormycosis in hematologic cancer patients.
Authors: Pagano L, Dragonetti G, de Carolis E, Veltri G, Del Principe MI, Busca A Abstract INTRODUCTION: Mucormycoses represent a rare but very aggressive class of mold infections occurring in patients with hematological malignancies (HMs). In the past, hematologic patients at high risk of invasive mucomycosis (IM) were those affected by acute myeloid leukemia but over the last ten years the prophylaxis with a very effective mold-active drug such as posaconazole, has completely modified the epidemiology. In fact, IM is now observed more frequently in patients with lymphoproliferative disorders who do not receive an...
Source: Expert Review of Hematology - July 17, 2020 Category: Hematology Tags: Expert Rev Hematol Source Type: research

Pharmacological management of rare coagulation factor deficiencies besides hemophilia.
Authors: Dorgalaleh A, Tabibian S, Hosseini MS, Shams M Abstract INTRODUCTION: Rare coagulation factor deficiencies are less-known disorders with variable effects on the patient's life. Management of such patients is a challenge due to the paucity of evidence-based data, more so when patients with these rare disorders encounter a more rare, related condition, like inhibitor development or thrombosis. AREA COVERED: A comprehensive literature search related to RCFDs and management was performed in PubMed in order to discuss therapeutic options and challenges, prophylaxis, management of minor and major surgeries, ...
Source: Expert Review of Hematology - July 17, 2020 Category: Hematology Tags: Expert Rev Hematol Source Type: research

An evaluation of subcutaneous daratumumab for the treatment of multiple myeloma.
Authors: Paul B, Atrash S, Bhutani M, Voorhees P, Hamadeh I, Usmani SZ Abstract INTRODUCTION: A subcutaneous formulation of daratumumab, a human immunoglobulin G1 kappa monoclonal antibody targeting CD38, recently achieved FDA approval for both newly diagnosed and relapsed refractory multiple myeloma amid promises to decrease infusion times and rates of infusion reactions in myeloma patients. AREAS COVERED: In this article the biology behind subcutaneous administration of oncologic antibody therapies is reviewed and the subcutaneous formulation of daratumumab is covered in depth. The most recent results from th...
Source: Expert Review of Hematology - July 15, 2020 Category: Hematology Tags: Expert Rev Hematol Source Type: research

Managing patients with hematological malignancies during COVID-19 pandemic.
Authors: Sahu KK, Cerny J PMID: 32580592 [PubMed - as supplied by publisher] (Source: Expert Review of Hematology)
Source: Expert Review of Hematology - June 27, 2020 Category: Hematology Tags: Expert Rev Hematol Source Type: research