MRI detection of brain abnormality in sickle cell disease.
Authors: Stotesbury H, Kawadler JM, Saunders DE, Kirkham FJ Abstract INTRODUCTION: Over the past decades, neuroimaging studies have clarified that a significant proportion of patients with sickle cell disease (SCD) have functionally significant brain abnormalities. Structural magnetic resonance imaging (MRI) sequences (T2, FLAIR, diffusion-weighted imaging) have been used by radiologists to diagnose chronic and acute cerebral infarction (both overt and clinically silent), whilst magnetic resonance angiography and venography have been used to diagnose arteriopathy and venous thrombosis. Imaging scientists have now d...
Source: Expert Review of Hematology - February 23, 2021 Category: Hematology Tags: Expert Rev Hematol Source Type: research

Voxelotor for the treatment of sickle cell disease.
Authors: Vissa M, Vichinsky E Abstract Introduction: The clinical manifestations of sickle cell disease (SCD) result from an inherited mutation in the beta-globin chain of hemoglobin (Hb) that causes Hb tetramers to polymerize when deoxygenated. The resulting erythrocyte deformation causes mechanical obstruction of blood flow by sickled cells, hemolysis, anemia and end-organ injury. While pain is the hallmark symptom of SCD, chronic organ injury as a result of anemia, inflammation and progressive vasculopathy play a major role in morbidity and mortality. Due to the complex pathophysiology of SCD, the need for an in...
Source: Expert Review of Hematology - February 20, 2021 Category: Hematology Tags: Expert Rev Hematol Source Type: research

Recent advances in surgery and its perioperative treatment in people with hemophilia.
Authors: Rodríguez-Merchán EC Abstract INTRODUCTION: People with hemophilia (PWH) may require some surgical procedures (orthopedic surgery, cardiac surgery, neurosurgery, et cetera) throughout their lives. AREAS COVERED: Articles on surgery and hemophilia published during the year 2020 were analyzed. The principal inclusion factor was a focal point on surgery in hemophilia. Articles that did not concentrate on this subject were not included. EXPERT OPINION: Turoctocog alfa pegol appears to be efficacious for perioperative hemostatic treatment of all kind of surgical operations (minor and major...
Source: Expert Review of Hematology - February 20, 2021 Category: Hematology Tags: Expert Rev Hematol Source Type: research

The important role of intensive induction chemotherapy in the treatment of acute myeloid leukemia.
Authors: Lin TL, Pagano L Abstract INTRODUCTION: Intensive induction chemotherapy followed by post-remission consolidation and/or allogeneic hematopoietic transplantation has been standard-of-care therapy for acute myeloid leukemia (AML) for decades. In recent years, a plethora of new agents have been approved for AML treatment, dramatically changing the AML treatment landscape. AREAS COVERED: This review provides an overview of the current role of intensive chemotherapy in the changing AML treatment landscape. PubMed-indexed publications (through 2020) and abstracts presented at major national and internationa...
Source: Expert Review of Hematology - February 19, 2021 Category: Hematology Tags: Expert Rev Hematol Source Type: research

Optimizing health-related quality of life in patients with chronic myeloid leukemia treated with tyrosine kinase inhibitors.
Authors: Efficace F, Vignetti M, Sparano F, Scalzulli E, Breccia M Abstract INTRODUCTION: The current treatment landscape of chronic myeloid leukemia (CML) is challenging for several reasons, and health-related quality of life (HRQOL) data may be of critical importance to help physicians and patients make more informed decisions. AREAS COVERED: A systematic literature search was performed in PubMed to identify the most recent studies (between April 2016 and June 2020) assessing the impact of tyrosine kinase inhibitors (TKIs) on adult CML patients' HRQOL. Studies assessing treatment discontinuation were also con...
Source: Expert Review of Hematology - February 10, 2021 Category: Hematology Tags: Expert Rev Hematol Source Type: research

Real world outcomes for the treatment of relapsed/refractory multiple myeloma patients with lenalidomide-dexamethasone combinations in a Latin American country. A retrospective cohort study from Grupo Argentino de Mieloma M últiple.
CONCLUSION: This is the first report of Rd combinations for the treatment of RRMM in Latin America. All combinations proved to be effective with an acceptable toxicity. PMID: 33541156 [PubMed - as supplied by publisher] (Source: Expert Review of Hematology)
Source: Expert Review of Hematology - February 7, 2021 Category: Hematology Tags: Expert Rev Hematol Source Type: research

Screening tools for hereditary hemolytic anemia: new concepts and strategies.
Authors: Fermo E, Vercellati C, Bianchi P Abstract INTRODUCTION: Hereditary haemolytic anemias are a group of rare and heterogeneous disorders due to abnormalities in structure, metabolism and transport functions of erythrocytes; they may overlap in clinical and haematological features making differential diagnosis difficult, particularly in mild and atypical forms. AREAS COVERED: In the present review the main tools currently adopted in routine hematologic investigation for the diagnosis of hereditary hemolytic anemias are described, together with the new diagnostic approaches that are being to be developed in...
Source: Expert Review of Hematology - February 7, 2021 Category: Hematology Tags: Expert Rev Hematol Source Type: research

Letter to the Editor: In response to: Richard F Pollock & Patrick Biggar. Indirect methods of comparison of the safety of ferric derisomaltose, iron sucrose and ferric carboxymaltose in the treatment of iron deficiency anemia In response to: Richard F Pollock & Patrick Biggar. Indirect methods of comparison of the safety of ferric derisomaltose, iron sucrose and ferric carboxymaltose in the treatment of iron deficiency anemia. Ex. Rev. Hem. 2020. https://doi.org/10.1080/17474086.2020.1709437.
Letter to the Editor: In response to: Richard F Pollock & Patrick Biggar. Indirect methods of comparison of the safety of ferric derisomaltose, iron sucrose and ferric carboxymaltose in the treatment of iron deficiency anemia In response to: Richard F Pollock & Patrick Biggar. Indirect methods of comparison of the safety of ferric derisomaltose, iron sucrose and ferric carboxymaltose in the treatment of iron deficiency anemia. Ex. Rev. Hem. 2020. https://doi.org/10.1080/17474086.2020.1709437. Expert Rev Hematol. 2021 Jan 29;: Authors: Aksan A, Dignass A, Stein J PMID: 33511892 [PubMed - as supplied...
Source: Expert Review of Hematology - January 31, 2021 Category: Hematology Tags: Expert Rev Hematol Source Type: research

Key questions in the new hemophilia era: update on concomitant use of FVIII and emicizumab in hemophilia A patients with inhibitors.
Authors: Carcao M, Mancuso ME, Young G, Jiménez-Yuste V Abstract Introduction: Immune tolerance induction (ITI) is the primary therapeutic strategy and only proven method to eradicate inhibitors to coagulation factor VIII (FVIII) in hemophilia A. Emicizumab, a humanized bispecific monoclonal antibody that mimics the function of activated FVIII, has expanded options to treat hemophilia A. The availability of emicizumab necessitates a revisit of recommendations for managing patients with inhibitors. Areas covered: Current evidence is reviewed about the concomitant use of emicizumab and FVIII concentrates durin...
Source: Expert Review of Hematology - January 28, 2021 Category: Hematology Tags: Expert Rev Hematol Source Type: research

Complement activation and coagulopathy - an ominous duo in COVID19.
Authors: Tomo S, Kumar KP, Roy D, Sankanagoudar S, Purohit P, Yadav D, Banerjee M, Sharma P, Misra S Abstract INTRODUCTION: COVID-19 has similarities to the Severe Acute Respiratory Syndrome (SARS) and Middle East Respiratory Syndrome (MERS) outbreaks, as severe patients and non-survivors have frequently shown abnormal coagulation profiles. Immune-mediated pathology is a key player in this disease; hence, the role of the complement system needs assessment. The complement system and the coagulation cascade share an intricate network, where multiple mediators maintain a balance between both pathways. Coagulopathy in ...
Source: Expert Review of Hematology - January 24, 2021 Category: Hematology Tags: Expert Rev Hematol Source Type: research

Venetoclax for the treatment of elderly or chemotherapy-ineligible patients with acute myeloid leukemia: a step in the right direction or a game changer?
Authors: Agarwal S, Kowalski A, Schiffer M, Zhao J, Bewersdorf JP, Zeidan AM Abstract INTRODUCTION: Acute myeloid leukemia (AML) is an aggressive malignancy with poor prognosis and high rates of relapse, especially in elderly patients who are ineligible to receive intensive chemotherapy. Venetoclax, an oral BCL-2 inhibitor, is approved by the Food and Drug Administration in combination with hypomethylating agents or low-dose cytarabine in newly-diagnosed AML patients who are ineligible to receive intensive chemotherapy. Confirmatory phase III VIALE-A and VIALE-C trials showed a composite complete remission rate of ...
Source: Expert Review of Hematology - January 21, 2021 Category: Hematology Tags: Expert Rev Hematol Source Type: research

Smoldering multiple myeloma 40 years later: a story of unintended disease.
Authors: Yavorkovsky LL Abstract INTRODUCTION: : Smoldering multiple myeloma (SMM) is a clonal plasma cell (PC) disorder considered a prelude to MM due to its greater malignant potential compared to monoclonal gammopathy of undetermined significance (MGUS). Despite tectonic changes in the SMM landscape that occurred since it was first distinguished four decades ago, SMM continues to represent a complex and controversial entity causing a great deal of diagnostic and management turmoil. AREAS COVERED: Author addresses increasingly complicated, ambiguous as well as some overlooked and misjudged aspects of SMM such...
Source: Expert Review of Hematology - January 14, 2021 Category: Hematology Tags: Expert Rev Hematol Source Type: research

A review of FDA-approved acute myeloid leukemia therapies beyond "7 + 3".
A review of FDA-approved acute myeloid leukemia therapies beyond "7 + 3". Expert Rev Hematol. 2021 Jan 11;: Authors: Bazinet A, Assouline S Abstract INTRODUCTION: The standard anthracycline and cytarabine-based chemotherapy for acute myeloid leukemia (AML) has changed relatively little since the 1970s and produces unsatisfactory outcomes in many patients. In the past two decades, a better understanding of the pathophysiology and heterogeneity of this disease has led to promising new therapies, resulting in a flurry of new drug approvals. AREAS COVERED: The MEDLINE database, ClinicalTrials...
Source: Expert Review of Hematology - January 14, 2021 Category: Hematology Tags: Expert Rev Hematol Source Type: research

Pregnancy-associated aplastic anemia: a case-based review.
Authors: Jaime-Pérez JC, González-Treviño M, Gómez-Almaguer D Abstract Introduction: Pregnancy-associated aplastic anemia (pAA) occurs when aplastic anemia (AA) is diagnosed for the first time during pregnancy and it is a rare but serious condition leading to severe maternal and fetal complications. It is unknown whether pregnancy triggers bone marrow failure or if this state is unrelated to the pathogenesis of pAA. Areas covered: In this review, three new cases of pAA are presented and its controversial etiologic relationship with pregnancy, its atypical presentation, and management are...
Source: Expert Review of Hematology - January 14, 2021 Category: Hematology Tags: Expert Rev Hematol Source Type: research

Association of tuberculosis in patients with chronic myeloid leukemia: a treatment proposal based on literature review.
Authors: Iqbal P, Soliman A, De Sanctis V, Yassin MA Abstract INTRODUCTION: Chronic Myeloid leukemia (CML) is one the first hematological malignancy linked with genetic alterations and have a targeted therapy with Tyrosine Kinase Inhibitors. However, there are certain unanswered questions, and many unmet needs which limit its treatment. Concurrent Mycobacterium Tuberculosis (Mtb) infection is one of those significant factors. Tuberculosis (TB) is a highly prevalent disease in association with diabetes mellitus, malignancy, poor socioeconomic environment, HIV, and other immunosuppressive conditions in developed and ...
Source: Expert Review of Hematology - January 14, 2021 Category: Hematology Tags: Expert Rev Hematol Source Type: research

Chronic lymphocytic leukemia prognostic models in real life: still a long way off.
Authors: Molica S PMID: 33438478 [PubMed - as supplied by publisher] (Source: Expert Review of Hematology)
Source: Expert Review of Hematology - January 14, 2021 Category: Hematology Tags: Expert Rev Hematol Source Type: research

A comprehensive overview of daratumumab and carfilzomib and the recently approved daratumumab, carfilzomib and dexamethasone regimen in relapsed/refractory multiple myeloma.
Authors: Richard S, Jagannath S, Cho HJ, Parekh S, Madduri D, Richter J, Chari A Abstract Introduction: Novel, effective regimens are needed in patients with relapsed and refractory myeloma (RRMM) who inevitably relapse after PI and IMID containing treatment. Areas covered: Pre-clinical data, early clinical and pivotal trials relevant to the development of the two backbone drugs of carfilzomib and daratumumab, and the two important recent trials, EQUULEUS and CANDOR leading to the FDA approval of the combination regimen of daratumumab, carfilzomib, and dexamethasone (DKd) for RRMM are detailed in this review. Exper...
Source: Expert Review of Hematology - December 19, 2020 Category: Hematology Tags: Expert Rev Hematol Source Type: research

The spectrum of beta-thalassemia mutations in the 22 Arab countries: a systematic review.
CONCLUSION: The findings of this study present a platform for further genetic epidemiological studies and will improve the genetic counseling for Arab patients with β-thal. PMID: 33317346 [PubMed - as supplied by publisher] (Source: Expert Review of Hematology)
Source: Expert Review of Hematology - December 16, 2020 Category: Hematology Tags: Expert Rev Hematol Source Type: research

An evaluation of ferric derisomaltose as a treatment for anemia.
Authors: Kassianides X, Bodington R, Bhandari S Abstract Introduction: Originally approved in Europe in 2009, ferric derisomaltose is the most recently authorized intravenous iron compound in the United States of America (2020). Ferric derisomaltose given as a rapid high-dose infusion can allow complete iron repletion in a single dose and it is now widely used in the treatment of iron deficiency. Areas covered: The chemistry, pharmacodynamics and pharmacokinetics of ferric derisomaltose are reviewed. Results from phase II, III and IV trials regarding efficacy and safety are presented. Mechanisms behind minor infusi...
Source: Expert Review of Hematology - December 16, 2020 Category: Hematology Tags: Expert Rev Hematol Source Type: research

Zanubrutinib for the treatment of Waldenstr öm Macroglobulinemia.
Zanubrutinib for the treatment of Waldenström Macroglobulinemia. Expert Rev Hematol. 2020 Dec 09;:1-8 Authors: Lim KJC, Tam CS Abstract Introduction: Waldenström Macroglobulinaemia (WM) is a heterogeneous, incurable condition which often relapses after chemoimmunotherapy. Novel therapies such as Bruton tyrosine-kinase (BTK) inhibitors have shown to be efficacious in treating WM but with an established, significant toxicity profile seen in the first-generation inhibitor Ibrutinib. Zanubrutinib is a selective, potent BTK inhibitor with the potential to reduce toxicity and improve efficacy. Area...
Source: Expert Review of Hematology - December 12, 2020 Category: Hematology Tags: Expert Rev Hematol Source Type: research

Biomarkers for acute and chronic graft versus host disease: state of the art.
Authors: Giaccone L, Faraci DG, Butera S, Lia G, Di Vito C, Gabrielli G, Cerrano M, Mariotti J, Dellacasa C, Felicetti F, Brignardello E, Mavilio D, Bruno B Abstract INTRODUCTION: Despite significant advances in treatment and prevention, graft-versus-host disease (GVHD) still represents the main cause of morbidity and mortality after allogeneic hematopoietic stem cell transplantation. Thus, considerable research efforts have been made to find and validate reliable biomarkers for diagnosis, prognosis and risk stratification of GVHD. AREAS COVERED: In this review the most recent evidences on different types of bi...
Source: Expert Review of Hematology - December 12, 2020 Category: Hematology Tags: Expert Rev Hematol Source Type: research

Hematopoietic cell transplantation during COVID-19 pandemic: experience from a tertiary hospital in Madrid.
Authors: Lázaro Del Campo P, Ramírez López A, de la Cruz Benito B, de Paz Arias R, de Soto Álvarez T, Sánchez Vadillo I, Humala Barbier K, García Pérez E, Dos Santos Ortas A, López de la Guía A, Gasior Kabat M, Baltasar Tello P, Jiménez Yuste V, Canales Albendea M PMID: 33280467 [PubMed - as supplied by publisher] (Source: Expert Review of Hematology)
Source: Expert Review of Hematology - December 9, 2020 Category: Hematology Tags: Expert Rev Hematol Source Type: research

Hemostatic defects in massive transfusion: an update and treatment recommendations.
Authors: Napolitano LM Abstract INTRODUCTION: Acute hemorrhage is a global healthcare issue, and remains the leading preventable cause of death in trauma. Acute severe hemorrhage can be related to traumatic, peripartum, gastrointestinal, and procedural causes. Hemostatic defects occur early in patients requiring massive transfusion. Early recognition and treatment of hemorrhage and hemostatic defects are required to save lives and to achieve optimal patient outcomes. AREAS COVERED: This review discusses current evidence and trials aimed at identifying the optimal treatment for hemostatic defects in hemorrhage a...
Source: Expert Review of Hematology - December 5, 2020 Category: Hematology Tags: Expert Rev Hematol Source Type: research

Evidence-based indications of platelet-rich plasma therapy.
Authors: Gupta S, Paliczak A, Delgado D Abstract INTRODUCTION: Platelet-rich plasma (PRP) is an autologous blood-derived product that contains platelet concentrations at least 2/3 times above the normal level and includes platelet-related growth factors. The concept of PRP began in the 1970s in the field of hematology to treat patients with thrombocytopenia. In the 1980s and 1990s, PRP began to be used in surgical procedures such as maxillofacial surgery and plastic surgery. Since then, PRP had been used in orthopedic procedures, cardiac surgery, sports injuries, plastic surgery, gynecology, urology, and more recen...
Source: Expert Review of Hematology - December 5, 2020 Category: Hematology Tags: Expert Rev Hematol Source Type: research

Chronic myelomonocytic leukemia - a review.
Authors: Thomopoulos TP, Bouhla A, Papageorgiou SG, Pappa V Abstract INTRODUCTION: Chronic myelomonocytic leukemia (CMML) is a clonal myeloid neoplasm, denoted by overlapping myelodysplastic and myeloproliferative features, with poor overall survival and high transformation rate to acute myeloid leukemia. AREAS COVERED: This review, following a thorough Medline search of pertinent published literature, discusses the diagnostic criteria, the pathogenesis, and the complex genetic landscape of the disease. Prognostication, response criteria, therapeutic management of patients, efficacy of established and novel tre...
Source: Expert Review of Hematology - December 5, 2020 Category: Hematology Tags: Expert Rev Hematol Source Type: research

Development of molecular intervention strategies for B-cell lymphoma.
Authors: Zhou W, Chen W Abstract INTRODUCTION: There are many genetic mutations involved in B-cell lymphomagenesis. These mutations contribute to the prognosis of B-cell lymphomas and can be used for diagnosis and targeted for intervention. AREAS COVERED: This review provides an overview on targeted gene therapies for B-cell lymphoma that were newly approved or are under clinical development. These include, TP53 mutations and related pathways, such as BTK inhibitors, MDM2/4 inhibitors, and XPO1 inhibitors; new drugs targeting EZH2 mutations through competitive inhibition, such as tazemetostat and GSK126; BCL-2-...
Source: Expert Review of Hematology - December 4, 2020 Category: Hematology Tags: Expert Rev Hematol Source Type: research

Romiplostim in adults with newly diagnosed or persistent immune thrombocytopenia.
Authors: Lozano ML, Godeau B, Grainger J, Matzdorff A, Rodeghiero F, Hippenmeyer J, Kuter DJ Abstract Introduction: Three distinct phases are recognized in immune thrombocytopenia (ITP): newly diagnosed (≤3 months after diagnosis), persistent (>3-12 months after diagnosis), and chronic (>12 months). Several international guidelines/expert recommendations have been released in the past 2 years regarding the treatment of newly diagnosed/persistent ITP. Areas covered: Across the guidelines/expert recommendations, thrombopoietin receptor agonists (TPO-RAs), including romiplostim (the focus ...
Source: Expert Review of Hematology - December 2, 2020 Category: Hematology Tags: Expert Rev Hematol Source Type: research

CD43 in the malignant flow cytometry laboratory in 2020.
CONCLUSION: CD43 has a growing role in the diagnosis and management of B cell malignancies; it has become essential for the classification of B cell lymphoproliferative disorders and may be of help in the differential diagnosis of CD10-positive lymphomas by FC. It is also required for optimal quantification of CLL residual disease, which will soon have therapeutic value. PMID: 33249940 [PubMed - as supplied by publisher] (Source: Expert Review of Hematology)
Source: Expert Review of Hematology - December 2, 2020 Category: Hematology Tags: Expert Rev Hematol Source Type: research

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