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    1436 results match your criteria Clinical Hematology[Journal]

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    Thrombopoietin and its receptor expression in pediatric patients with chronic immune thrombocytopenia.
    Hematology 2018 Jan 9:1-6. Epub 2018 Jan 9.
    a Department of Hematology and Oncology , Shanghai Children's Hospital, Shanghai Jiao Tong University , Shanghai , People's Republic of China.
    Objectives: Chronic immune thrombocytopenia (cITP) is common in children. However, the pathogenesis has not been fully elucidated. This study aimed to determine whether thrombopoietin (TPO) and its receptor c-mannosylation of the TPO receptor (c-Mpl) have an impact on childhood cITP. Read More

    The potential association of CMV-specific CD8+ T lymphocyte reconstitution with the risk of CMV reactivation and persistency in post allogeneic stem cell transplant patients.
    Hematology 2018 Jan 9:1-7. Epub 2018 Jan 9.
    d Clinical and Chemical Pathology, Egyptian Anti Doping Lab , International Medical Center , Cairo , Egypt.
    Objectives: development of cytomegalovirus (CMV)-specific CD8+ T cell response is crucial in preventing symptomatic CMV infection specially, in stem cell transplant (SCT) patients. The aim of this study was to evaluate CMV-specific CD8+ T cell reconstitution in allogeneic SCT recipients and to study the possible association between CMV-specific CD8+ T cell recovery with protection from CMV reactivation and persistency.

    Methods: Human leuKocyte antigen (HLA)-tetramers were used for CMV-specific CD8+ cell quantitation by Flow cytometry in twenty post-allogeneic SCT patients. Read More

    Is there a difference in phenotype between males and females with non-transfusion-dependent thalassemia? A cross-sectional evaluation.
    Hematology 2018 Jan 5:1-4. Epub 2018 Jan 5.
    c UOSD Malattie Rare del Globulo Rosso , AORN A. Cardarelli , Naples , Italy.
    Objectives: Non-transfusion-dependent thalassemia includes a variety of phenotypes and genotypes that rarely require regular transfusions. However, these patients can experience a wide range of complications. The objective of this retrospective study was to verify whether there is a significant difference in non-transfusion-dependent thalassemia-related complications and treatment among males and females. Read More

    Eltrombopag in the management of aplastic anaemia: real-world experience in a non-trial setting.
    Hematology 2018 Jan 5:1-6. Epub 2018 Jan 5.
    a Department of Medicine , Queen Mary Hospital , Hong Kong.
    Objective: The thrombopoietin mimetic eltrombopag has been used in clinical trials for the frontline and salvage treatment of aplastic anaemia (AA). Eltrombopag was investigated in AA patients on a non-trial all-comer basis.

    Methods: Consecutive newly diagnosed and relapsed/refractory AA patients were treated with eltrombopag. Read More

    The clinical significance of FLT3 ITD mutation on the prognosis of adult acute promyelocytic leukemia.
    Hematology 2017 Dec 18:1-6. Epub 2017 Dec 18.
    a Department of Laboratory Diagnosis , Shidong Hospital of Yangpu Distric , Shanghai , People's Republic of China.
    Background And Aims: To explore the relationship between FLT3 (encoding Fms related tyrosine kinase 3) internal tandem duplication (ITD) mutations with the prognosis of acute promyelocytic leukemia. The PubMed database, the Cochrane Library, conference proceedings, the EMBASE databases, and references of published trials and review articles were searched. Two reviewers independently assessed the quality of the trials and extracted the data. Read More

    Understanding of the significance and health implications of asplenia in a cohort of patients with haemaglobinopathy: possible benefits of a spleen registry.
    Hematology 2017 Dec 13:1-5. Epub 2017 Dec 13.
    a Faculty of Medicine, Nursing and Health Sciences , Monash University , Clayton , Australia.
    Objectives: Asplenia and hyposplenism carry a significant risk of ongoing morbidity and mortality which can be reduced by education, vaccination and antibiotic use. We aimed to assess education and other methods of prevention in a cohort of patients with haemoglobinopathy in a tertiary referral centre, which also had access to a post-splenectomy registry created to reduce post-splenectomy infection risk.

    Methods: A standardized questionnaire was used on patients who attended the service for regular therapy. Read More

    Inferior vena cava filter use and patient safety: legacy or science?
    Hematology Am Soc Hematol Educ Program 2017 Dec;2017(1):686-692
    Thromboembolism Program, Sunnybrook Health Sciences Centre, Toronto, ON, Canada.
    There has been a dramatic increase in vena cava filter (VCF) use over the past 20 years in the absence of evidence that filters provide a net patient benefit or are required in most cases. This increase is largely attributable to the availability of retrievable filters and expanded indications, particularly as primary prophylaxis in patients thought to be at high risk of pulmonary embolism. Substantial variability in VCF use, unrelated to patient clinical factors, has been shown between hospitals, from region to region, and among various countries. Read More

    Assessing thrombocytopenia in the intensive care unit: the past, present, and future.
    Hematology Am Soc Hematol Educ Program 2017 Dec;2017(1):660-666
    Division of Critical Care, Department of Internal Medicine, University of Manitoba, Winnipeg, MB, Canada; and.
    Thrombocytopenia is common among patients admitted to the intensive care unit (ICU). Multiple pathophysiological mechanisms may contribute, including thrombin-mediated platelet activation, dilution, hemophagocytosis, extracellular histones, ADAMTS13 deficiency, and complement activation. From the clinical perspective, the development of thrombocytopenia in the ICU usually indicates serious organ system derangement and physiologic decompensation rather than a primary hematologic disorder. Read More

    Plasma exchange in thrombotic microangiopathies (TMAs) other than thrombotic thrombocytopenic purpura (TTP).
    Hematology Am Soc Hematol Educ Program 2017 Dec;2017(1):632-638
    Therapeutic Apheresis Treatment Unit, Division of Transfusion Medicine, Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, MN.
    Thrombotic microangiopathies (TMAs) are a diverse group of disorders that are characterized by common clinical and laboratory features. The most commonly thought-of TMA is thrombotic thrombocytopenic purpura (TTP). Because of the marked improvement in patient mortality associated with the use of therapeutic plasma exchange (TPE) in TTP, this therapy has been applied to all of the TMAs. Read More

    Developing T-cell therapies for lymphoma without receptor engineering.
    Hematology Am Soc Hematol Educ Program 2017 Dec;2017(1):622-631
    Center for Cancer and Immunology Research, Children's National Health System, Washington, DC; and.
    T-cell therapy has emerged from the bench for the treatment of patients with lymphoma. Responses to T-cell therapeutics are regulated by multiple factors, including the patient's immune system status and disease stage. Outside of engineering of chimeric antigen receptors and artificial T-cell receptors, T-cell therapy can be mediated by ex vivo expansion of antigen-specific T cells targeting viral and/or nonviral tumor-associated antigens. Read More

    Harnessing the power of the immune system in non-Hodgkin lymphoma: immunomodulators, checkpoint inhibitors, and beyond.
    Hematology Am Soc Hematol Educ Program 2017 Dec;2017(1):618-621
    Mayo Clinic, Rochester, MN.
    Non-Hodgkin lymphoma is a malignancy of B lymphocytes that typically infiltrate sites of disease, including the lymph nodes, spleen, and bone marrow. Beyond the presence of malignant cells, many immune cells are also present within the tumor microenvironment. Although these immune cells have the potential to regulate the growth of malignant B cells, intratumoral immune cells are unable to eradicate lymphoma cells and most patients with lymphoma have clinical evidence of disease progression. Read More

    Novel alternate hemostatic agents for patients with inhibitors: beyond bypass therapy.
    Hematology Am Soc Hematol Educ Program 2017 Dec;2017(1):605-609
    Department of Medicine, Division Hematology/Oncology, University of Pittsburgh, and Hemophilia Center of Western Pennsylvania, Pittsburgh, PA.
    Inhibitor formation is among the most severe complications of hemophilia treatment. With a cumulative incidence of ∼30% in those with severe hemophilia A and ∼3% in those with severe hemophilia B, inhibitors are caused by a T-cell response directed against infused coagulation factor; these inhibitors neutralize factor VIII or IX activity and disrupt normal hemostasis. Inhibitor patients become unresponsive to standard factor treatment and, as an alternative, use bypass treatment (eg, recombinant factor VIIa or factor VIII inhibitor bypass activity). Read More

    Using pharmacokinetics to individualize hemophilia therapy.
    Hematology Am Soc Hematol Educ Program 2017 Dec;2017(1):595-604
    Department of Health Research, Methods, Evidence, and Impact, and.
    Prevention and treatment of bleeding in hemophilia requires that plasma clotting factor activity of the replaced factor exceeds a defined target level. Most clinical decisions in hemophilia are based on implicit or explicit application of pharmacokinetic measures. The large interindividual variability in pharmacokinetics of factor concentrates suggests that relying on the average pharmacokinetic characteristics of factor concentrates would not allow optimizing the treatment of individual patients; for example, adjusting the frequency of infusions and targeting a specific clotting factor activity level on a case-by-case basis. Read More

    Hemophilia gene therapy comes of age.
    Hematology Am Soc Hematol Educ Program 2017 Dec;2017(1):587-594
    Division of Pediatrics, The Children's Hospital of Philadelphia, Philadelphia, PA; and.
    Concurrent with the development of recombinant factor replacement products, the characterization of the F9 and F8 genes over 3 decades ago allowed for the development of recombinant factor products and made the hemophilias a target disease for gene transfer. The progress of hemophilia gene therapy has been announced in 3 American Society of Hematology scientific plenary sessions, including the first "cure" in a large animal model of hemophilia B in 1998, first in human sustained vector-derived factor IX activity in 2011, and our clinical trial results reporting sustained vector-derived factor IX activity well into the mild or normal range in 2016. This progression to clinically meaningful success combined with numerous ongoing recombinant adeno-associated virus (rAAV)-mediated hemophilia gene transfer clinical trials suggest that the goal of gene therapy to alter the paradigm of hemophilia care may soon be realized. Read More

    Secondary CNS relapse in diffuse large B-cell lymphoma: defining high-risk patients and optimization of prophylaxis strategies.
    Hematology Am Soc Hematol Educ Program 2017 Dec;2017(1):578-586
    Department of Medical Oncology, British Columbia Cancer Agency, Vancouver, BC, Canada.
    Despite improvement in survival in diffuse large B-cell lymphoma (DLBCL) with the introduction of rituximab, central nervous system (CNS) relapse continues to represent a clinical challenge. A number of studies have evaluated clinical risk factors in an attempt to identify high-risk patients to direct CNS staging investigations and consider prophylaxis strategies. The CNS International Prognostic Index is a robust and reproducible risk model that can identity patients at high risk of CNS relapse, but its specificity remains limited. Read More

    Biology of CNS lymphoma and the potential of novel agents.
    Hematology Am Soc Hematol Educ Program 2017 Dec;2017(1):556-564
    Division of Hematology/Oncology, University of California, San Francisco, San Francisco, CA.
    Primary and secondary CNS lymphomas are aggressive brain tumors that pose an immense challenge to define in terms of molecular pathogenesis, as well as to effectively treat. During the past 10 years improvements in survival have been achieved with the implementation of anti-CD20 immunotherapy and optimization of dose-intensive consolidation strategies. The applications of whole-exome sequencing, comparative genomic hybridization, transcriptional profiling, and examination of the tumor microenvironment, particularly in the context of clinical investigation, provide insights that create a roadmap for the development and implementation of novel targeted agents for this disease. Read More

    Pain-measurement tools in sickle cell disease: where are we now?
    Hematology Am Soc Hematol Educ Program 2017 Dec;2017(1):534-541
    Section of Pediatric Hematology/Oncology, Medical College of Wisconsin, Milwaukee, WI; and.
    Pain is a complex multidimensional experience and the most common morbidity in patients with sickle cell disease (SCD). Tools to assess pain can be of use not only to guide pain treatment but also to provide insight into underlying pain neurobiology. Mechanisms of pain in SCD are multifactorial and are not completely elucidated. Read More

    Optimizing the care model for an uncomplicated acute pain episode in sickle cell disease.
    Hematology Am Soc Hematol Educ Program 2017 Dec;2017(1):525-533
    Departments of Haematology and Paediatric Haematology, Royal London Hospital, Bart's Health National Health Service Trust, London, United Kingdom.
    The pathophysiology, clinical presentation, and natural history of acute pain in sickle cell disease are unique and require a disease-centered approach that also applies general principles of acute and chronic pain management. The majority of acute pain episodes are managed at home without the need to access health care. The long-term consequences of poorly treated acute pain include chronic pain, adverse effects of chronic opioid usage, psychological maladjustment, poor quality of life, and excessive health care utilization. Read More

    Emerging options in multiple myeloma: targeted, immune, and epigenetic therapies.
    Hematology Am Soc Hematol Educ Program 2017 Dec;2017(1):518-524
    Division of Hematology and Internal Medicine, Mayo Clinic, Rochester, MN.
    Considerable progress has been made in the treatment of multiple myeloma in the past decade with median survival for the disease improving significantly. This has come through a combination of better understanding of the disease biology and coordinated research into new treatment approaches including better supportive care. However, patients eventually become refractory to available treatments and succumb to the disease, highlighting the need to develop new treatment approaches. Read More

    Management of multiple myeloma in the relapsed/refractory patient.
    Hematology Am Soc Hematol Educ Program 2017 Dec;2017(1):508-517
    Department of Hematology, Erasmus Medical Center Cancer Institute, Rotterdam, The Netherlands.
    The approach to the patient with relapsed or relapsed/refractory multiple myeloma requires a careful evaluation of the results of previous treatments, the toxicities associated with it, and an assessment of prognostic factors. The majority of patients will have received prior therapy with drug combinations, including a proteasome inhibitor and an immune-modulatory agent. It is the physician's task to choose the right moment for the start of therapy and decide with the patient which goals need to be achieved. Read More

    Management of multiple myeloma in the newly diagnosed patient.
    Hematology Am Soc Hematol Educ Program 2017 Dec;2017(1):498-507
    Clínica Universidad de Navarra, Centro de Investigación Médica Aplicada, Navarra, Spain.
    Multiple myeloma is the second most frequent hematological disease. The introduction of melphalan as high-dose therapy followed by autologous hematopoietic cell transplantation (HDT/ASCT) for young patients and the availability of novel agents for young and elderly patients with multiple myeloma have dramatically changed the perspective of treatment. However, further research is necessary if we want definitively to cure the disease. Read More

    What are the current treatment approaches for patients with polycythemia vera and essential thrombocythemia?
    Hematology Am Soc Hematol Educ Program 2017 Dec;2017(1):480-488
    Center of Research and Innovation of Myeloproliferative Neoplasms (CRIMM), Careggi University Hospital/University of Florence, Florence, Italy.
    Polycythemia vera (PV) and essential thrombocythemia (ET) are chronic myeloproliferative neoplasms that are characterized by thrombohemorrhagic complications, symptom burden, and impaired survival mainly due to thrombosis, progression to myelofibrosis, and transformation to acute leukemia. In this manuscript, we will review the most recent changes in diagnostic criteria, the improvements in risk stratification, and the "state of the art" in the daily management of these disorders. The role of conventional therapies and novel agents, interferon α and the JAK2 inhibitor ruxolitinib, is critically discussed based on the results of a few basic randomized clinical studies. Read More

    Myeloproliferative neoplasms: from origins to outcomes.
    Hematology Am Soc Hematol Educ Program 2017 Dec;2017(1):470-479
    Department of Haematology, Cambridge Institute for Medical Research and Wellcome Trust/MRC Stem Cell Institute, University of Cambridge, United Kingdom; and.
    Substantial progress has been made in our understanding of the pathogenetic basis of myeloproliferative neoplasms. The discovery of mutations in JAK2 over a decade ago heralded a new age for patient care as a consequence of improved diagnosis and the development of therapeutic JAK inhibitors. The more recent identification of mutations in calreticulin brought with it a sense of completeness, with most patients with myeloproliferative neoplasm now having a biological basis for their excessive myeloproliferation. Read More

    Incorporating novel approaches in the management of MDS beyond conventional hypomethylating agents.
    Hematology Am Soc Hematol Educ Program 2017 Dec;2017(1):460-469
    Department of Medicine, Section of Hematology/Oncology, The University of Chicago, Chicago, IL; and The University of Chicago Comprehensive Cancer Center, Chicago, IL.
    In the last decade, the treatment of higher-risk myelodysplastic syndromes (MDS) has revolved around the azanucleosides, azacitidine and decitabine, which at lower doses are postulated to work predominantly via their effects on inhibition of DNA methyltransferases and consequent DNA hypomethylation. For patients who relapse after, or do not respond to, hypomethylating agent therapy, the outcome is dismal, and new agents and approaches that have the potential to alter the natural history of these diseases are desperately needed. Allogeneic stem cell transplant is the only known potentially curative approach in MDS, but its applicability has been limited by the advanced age of patients and attendant comorbidities. Read More

    Current treatment algorithm for the management of lower-risk MDS.
    Hematology Am Soc Hematol Educ Program 2017 Dec;2017(1):453-459
    Department of Hematology, Oncology and Palliative Care, Marien Hospital Düsseldorf, Düsseldorf, Germany.
    Lower risk myelodysplastic syndromes (MDS), defined as MDS with a Revised International Prognostic Scoring System score ≤3.5 points, will remain a challenging entity in 2018. Supportive care continues to be the linchpin of treatment, although the options to reduce transfusion needs are broadening. Read More

    Uncoding the genetic heterogeneity of myelodysplastic syndrome.
    Hematology Am Soc Hematol Educ Program 2017 Dec;2017(1):447-452
    Department of Medical Oncology, Dana-Farber Cancer Institute, Boston, MA.
    Myelodysplastic syndrome (MDS) is a clinically heterogeneous disease characterized by functional impairment of hematopoiesis and abnormal bone marrow morphology. The type and severity of hematopoietic dysfunction in MDS are highly variable, and the kinetics of disease progression are difficult to predict. Genomic studies have shown that MDS is typically driven by a multistep somatic genetic process affecting a core set of genes. Read More

    Chronic organ failure in adult sickle cell disease.
    Hematology Am Soc Hematol Educ Program 2017 Dec;2017(1):435-439
    UCSF Benioff Children's Hospital Oakland, Oakland, CA.
    Sickle cell disease is now a chronic adult illness characterized by progressive multiorgan failure, particularly involving the brain and kidney. The etiology is multifactorial; it includes hemolysis and nitric oxide deficiency. As patients age, most experience neurologic insult. Read More

    Hereditary thrombocytopenias: a growing list of disorders.
    Hematology Am Soc Hematol Educ Program 2017 Dec;2017(1):385-399
    Department of Internal Medicine, IRCCS Policlinico San Matteo Foundation and University of Pavia, Pavia, Italy.
    The introduction of high throughput sequencing (HTS) techniques greatly improved the knowledge of inherited thrombocytopenias (ITs) over the last few years. A total of 33 different forms caused by molecular defects affecting at least 32 genes have been identified; along with the discovery of new disease-causing genes, pathogenetic mechanisms of thrombocytopenia have been better elucidated. Although the clinical picture of ITs is heterogeneous, bleeding has been long considered the major clinical problem for patients with IT. Read More

    Improved biological insight and influence on management in indolent lymphoma. Talk 3: update on nodal and splenic marginal zone lymphoma.
    Hematology Am Soc Hematol Educ Program 2017 Dec;2017(1):371-378
    Hemato-Oncology, Hôpital Saint-Louis, Assistance Publique-Hôpitaux de Paris, Paris, France; Diderot University, Sorbonne Paris-Cité, Paris, France; and EA7324, Descartes University, Paris, France.
    Splenic marginal zone lymphoma (SMZL) and nodal marginal zone lymphoma (NMZL) are rare indolent chronic B-cell lymphomas. Prognosis is typically good with median survival around 10-15 years. Management is generally based on the presence of symptoms or high tumor burden. Read More

    Toward personalized treatment in Waldenström macroglobulinemia.
    Hematology Am Soc Hematol Educ Program 2017 Dec;2017(1):365-370
    Bing Center for Waldenström Macroglobulinemia, Dana-Farber Cancer Institute, Harvard Medical School, Boston, MA.
    Waldenström macroglobulinemia (WM) is a rare lymphoma with 1000 to 1500 new patients diagnosed per year in the United States. Patients with WM can experience prolonged survival times, which seem to have increased in the last decade, but relapse is inevitable. The identification of recurrent mutations in the MYD88 and CXCR4 genes has opened avenues of research to better understand and treat patients with WM. Read More

    Follicular lymphoma: are we ready for a risk-adapted approach?
    Hematology Am Soc Hematol Educ Program 2017 Dec;2017(1):358-364
    Division of Oncology, Washington University School of Medicine, St. Louis, MO.
    Follicular lymphoma is the most common indolent non-Hodgkin lymphoma in the Western hemisphere. The natural history of FL appears to have been favorably impacted by the introduction of rituximab after randomized clinical trials demonstrated that the addition of rituximab to standard chemotherapy induction has improved the overall survival. Yet, the disease is biologically and clinically heterogeneous with wide variations in outcomes for individual patients. Read More

    How should we sequence and combine novel therapies in CLL?
    Hematology Am Soc Hematol Educ Program 2017 Dec;2017(1):346-353
    Department of Medical Oncology, Dana-Farber Cancer Institute, Boston, MA.
    With the recent approval of several effective and well-tolerated novel agents (NAs), including ibrutinib, idelalisib, venetoclax, and obinutuzumab, patients with chronic lymphocytic leukemia (CLL) have more therapeutic options than ever before. The availability of these agents is both an important advance for patients but also a challenge for practicing hematologist/oncologists to learn how best to sequence NAs, both with respect to chemoimmunotherapy (CIT) and to other NAs. The sequencing of NAs in clinical practice should be guided both by an individual patient's prognostic markers, such as FISH and immunoglobulin heavy chain variable region (IGHV)-mutation status, as well as the patient's medical comorbidities and goals of care. Read More

    Optimizing frontline therapy of CLL based on clinical and biological factors.
    Hematology Am Soc Hematol Educ Program 2017 Dec;2017(1):338-345
    Department I of Internal Medicine, Center of Integrated Oncology Cologne Bonn, University of Cologne, Cologne, Germany; and.
    The heterogeneity of the clinical course of chronic lymphocytic leukemia (CLL) ranges from an indolent course, where patients do not require therapy for many years, to a very aggressive disease, where treatment is required soon after diagnosis and relapses may occur early. The improved tools for prognostication allow predicting the outcome of patients with increasing reliability. Some markers also allow selecting more specific therapies with improved activity in the presence of certain genetic or clinical features of CLL. Read More

    The mutational landscape of chronic lymphocytic leukemia and its impact on prognosis and treatment.
    Hematology Am Soc Hematol Educ Program 2017 Dec;2017(1):329-337
    Hematology, Oncology Institute of Southern Switzerland, Bellinzona, Switzerland; and.
    The typical genome of chronic lymphocytic leukemia (CLL) carries ∼2000 molecular lesions. Few mutations recur across patients at a frequency >5%, whereas a large number of biologically and clinically uncharacterized genes are mutated at lower frequency. Approximately 80% of CLL patients carry at least 1 of 4 common chromosomal alterations, namely deletion 13q14, deletion 11q22-23, deletion 17p12, and trisomy 12. Read More

    Emerging role of novel therapies in Hodgkin lymphoma: proceed with caution.
    Hematology Am Soc Hematol Educ Program 2017 Dec;2017(1):317-323
    Washington University School of Medicine, Siteman Cancer Center, St. Louis, MO.
    Based on very high response rates in the relapsed and refractory setting, brentuximab vedotin and the programmed cell death protein 1 (PD-1) inhibitors, nivolumab and pembrolizumab, have quickly been incorporated into clinical trials for first- and second-line therapy of Hodgkin lymphoma. Preliminary data show that brentuximab vedotin alone is not adequate therapy for newly diagnosed Hodgkin lymphoma in older patients, but modestly decreases the risk of relapse when combined with adriamycin, vinblastine, and dacarbazine in patients with previously untreated advanced-stage disease. In second-line therapy, combining brentuximab vedotin with conventional chemotherapy or with PD-1 inhibitors as pretransplant salvage is associated with high overall and complete response rates, although further follow up is needed to assess whether posttransplant outcomes are improved. Read More

    Optimizing therapy for mantle cell lymphoma.
    Hematology Am Soc Hematol Educ Program 2017 Dec;2017(1):304-309
    Department of Medicine, Division of Hematology-Medical Oncology, Weill Cornell Medicine, New York, NY.
    Most people with mantle cell lymphoma (MCL) present with diffuse adenopathy and benefit from early initiation of rituximab and high-dose cytarabine- or bendamustine-based therapies. Some patients, however, present with primarily nonnodal disease that can follow either an indolent or a rapidly progressive, treatment-resistant clinical course. Rarely, patients present with explosive disease that can be challenging to manage and often involves the central nervous system. Read More

    Primary mediastinal B-cell lymphoma: biology and evolving therapeutic strategies.
    Hematology Am Soc Hematol Educ Program 2017 Dec;2017(1):298-303
    George Washington University, Washington, DC; and.
    Primary mediastinal B-cell lymphoma (PMBCL) is recognized as a distinct clinicopathologic entity that predominantly affects adolescents and young adults and is more common in female subjects. Although PMBCL is considered to be a subtype of diffuse large B-cell lymphoma, its clinical, morphologic, and biological characteristics overlap significantly with those of nodular sclerosing Hodgkin lymphoma (NSHL). Over the past few years, the shared biology of these 2 entities has been highlighted in several studies, and mediastinal gray zone lymphoma, with features intermediate between PMBCL and NSHL, has been recognized as a unique molecular entity. Read More

    Tailoring front-line therapy in diffuse large B-cell lymphoma: who should we treat differently?
    Hematology Am Soc Hematol Educ Program 2017 Dec;2017(1):284-294
    Cancer Research UK Centre, Cancer Sciences Unit, Faculty of Medicine, University of Southampton, Southampton General Hospital, Southampton, United Kingdom.
    Although there have been significant insights into the biology of diffuse large B-cell lymphoma (DLBCL) over recent years, progress in our therapeutic approach has been disappointing over the same timeframe. This is not for want of trying. In 2017, R-CHOP (rituximab plus cyclophosphamide, doxorubicin, vincristine, and prednisone) remains the "gold standard," despite all of our insights into cell-of-origin and other subgroups. Read More

    New therapeutic targets in transfusion-dependent and -independent thalassemia.
    Hematology Am Soc Hematol Educ Program 2017 Dec;2017(1):278-283
    Fondazione IRCCS, Cà Granda Policlinico, Milan, Italy; and.
    β-Thalassemias are characterized by reduced production of β-globin chain, resulting in α/β-chain unbalance and precipitation of α-globin-heme complexes and determining ineffective erythropoiesis. Ineffective erythropoiesis, chronic hemolytic anemia, and compensatory hematopoietic expansion are the disease hallmarks, and they are related to the severity of the chain unbalance. Several clinical forms of β-thalassemia, including the coinheritance of β-thalassemia with hemoglobin E resulting in hemoglobin E/β-thalassemia, have been described. Read More

    Impact of bone disease and pain in thalassemia.
    Hematology Am Soc Hematol Educ Program 2017 Dec;2017(1):272-277
    Department of Clinical and Biological Sciences, University of Torino, Torino, Italy.
    Conventional treatment of thalassemia, namely regular blood transfusion and iron chelation, improves perspectives and quality of life; however, successful treatment leads to more time in which long-term complications such as bone disease can develop. Thalassemia bone disease (TBD) is unique: all aspects, from bone anatomy and bone quality to mineral density, may be affected, with important morbidity including osteoporosis, fractures, spinal deformities, nerve compression, and pain. Clinical presentations include growth impairment, rickets-like features, back pain, spinal deformities, any sign of nerve compression, severe osteoporosis, and fragility fractures. Read More

    Iron overload in thalassemia: different organs at different rates.
    Hematology Am Soc Hematol Educ Program 2017 Dec;2017(1):265-271
    Department of Medicine, Indiana University School of Medicine, Indianapolis, IN.
    Thalassemic disorders lie on a phenotypic spectrum of clinical severity that depends on the severity of the globin gene mutation and coinheritance of other genetic determinants. Iron overload is associated with increased morbidity in both patients with transfusion-dependent thalassemia (TDT) and non-transfusion-dependent thalassemia (NTDT). The predominant mechanisms driving the process of iron loading include increased iron burden secondary to transfusion therapy in TDT and enhanced intestinal absorption secondary to ineffective erythropoiesis and hepcidin suppression in NTDT. Read More

    Incorporation of nonchemotherapeutic agents in pediatric acute lymphoblastic leukemia.
    Hematology Am Soc Hematol Educ Program 2017 Dec;2017(1):259-264
    Dana-Farber Cancer Institute/Boston Children's Hospital, Boston, MA.
    With current available therapies, the prognosis for most children and adolescents with acute lymphoblastic leukemia (ALL) is favorable. However, the multiagent chemotherapy regimens used to treat newly diagnosed patients are associated with many acute and long-term complications, and therapy for relapsed disease is intensive and suboptimally effective. Over the last decade, several nonchemotherapeutic approaches have been evaluated, with the goal of identifying more effective, less toxic therapies that can be used in conjunction with, or even replace, current regimens. Read More

    Crisis management in the treatment of childhood acute lymphoblastic leukemia: putting right what can go wrong (emergency complications of disease and treatment).
    Hematology Am Soc Hematol Educ Program 2017 Dec;2017(1):251-258
    Department of Haematology, Great Ormond Street Hospital for Children, London, United Kingdom.
    The improvement in overall survival in children with acute lymphoblastic leukemia (ALL) over the last 5 decades has been considerable, with around 90% now surviving long term. The risk of relapse has been reduced to such an extent that the risk of treatment-related mortality is now approaching that of mortality caused by relapse. Toxicities may also lead to the suboptimal delivery of chemotherapy (treatment delays, dose reductions, dose omissions), potentially increasing relapse risk, and short- and long-term morbidity, adding to the "burden of therapy" in an increasing number of survivors. Read More

    Pediatric leukemia susceptibility disorders: manifestations and management.
    Hematology Am Soc Hematol Educ Program 2017 Dec;2017(1):242-250
    Clinical Genetics Branch, Division of Cancer Epidemiology and Genetics, National Cancer Institute, Bethesda, MD.
    The clinical manifestations of inherited susceptibility to leukemia encompass a wide phenotypic range, including patients with certain congenital anomalies or early-onset myelodysplastic syndrome (MDS) and some with no obvious medical problems until they develop leukemia. Leukemia susceptibility syndromes occur as a result of autosomal dominant, autosomal recessive, or X-linked recessive inheritance, or de novo occurrence, of germline pathogenic variants in DNA repair, ribosome biogenesis, telomere biology, hematopoietic transcription factors, tumor suppressors, and other critical cellular processes. Children and adults with cytopenias, MDS, dysmorphic features, notable infectious histories, immunodeficiency, certain dermatologic findings, lymphedema, unusual sensitivity to radiation or chemotherapy, or acute leukemia with a family history of early-onset cancer, pulmonary fibrosis, or alveolar proteinosis should be thoroughly evaluated for a leukemia susceptibility syndrome. Read More

    Symptomatic subsegmental pulmonary embolism: to treat or not to treat?
    Hematology Am Soc Hematol Educ Program 2017 Dec;2017(1):237-241
    Department of Thrombosis and Hemostasis, Leiden University Medical Center, Leiden, The Netherlands.
    The introduction of computed tomographic pulmonary angiography and its recent increasing availability has led to a significant rise in its use to help clinicians diagnose acute pulmonary embolism (PE). This has led to a significant increase in the incidence of PE diagnoses. Simultaneously, the case fatality rate of acute PE has been decreasing and no significant change in its mortality has been noted, suggesting that the additional PE diagnoses are less severe and these patients might not benefit from anticoagulation therapy. Read More

    Controversies in venous thromboembolism: to treat or not to treat superficial vein thrombosis.
    Hematology Am Soc Hematol Educ Program 2017 Dec;2017(1):223-230
    Thrombosis Research Unit, Division of Hematology, Department of Medicine I, Carl Gustav Carus University Hospital, Technische Universität Dresden, Dresden, Germany; and King's Thrombosis Service, Department of Hematology, King's College London, London, United Kingdom.
    The management of superficial vein thrombosis (SVT) is poorly defined and remains controversial overall. SVT has long been considered a benign, self-limited disease, but recent studies show that SVT carries a nonnegligible risk for recurrence, deep vein thrombosis, or pulmonary embolism. Current guidelines recommend the use of low-molecular-weight heparin or fondaparinux, but results of several surveys indicate that the majority of patients with SVT receive nonanticoagulant therapy only, which includes compression stockings or bandages, nonsteroidal anti-inflammatory drugs, topical application of heparin gel, or surgical interventions. Read More

    Shall we treat smoldering multiple myeloma in the near future?
    Hematology Am Soc Hematol Educ Program 2017 Dec;2017(1):194-204
    Myeloma Service, Department of Medicine, Memorial Sloan Kettering Cancer Center, New York, NY.
    In recent years, several new drugs have been approved for the treatment of multiple myeloma. Many of these newer drugs are highly efficacious and less toxic than older chemotherapy drugs. In 2014, the diagnostic criteria for multiple myeloma were revised. Read More

    New monogenic disorders identify more pathways to neutropenia: from the clinic to next-generation sequencing.
    Hematology Am Soc Hematol Educ Program 2017 Dec;2017(1):172-180
    Department of Pediatrics, Massey Cancer Center, Virginia Commonwealth University, Richmond, VA.
    Neutrophils are the most common type of leukocyte in human circulating blood and constitute one of the chief mediators for innate immunity. Defined as a reduction from a normal distribution of values, neutropenia results from a number of congenital and acquired conditions. Neutropenia may be insignificant, temporary, or associated with a chronic condition with or without a vulnerability to life-threatening infections. Read More

    Diagnosis and management of postpartum ovarian vein thrombosis.
    Hematology Am Soc Hematol Educ Program 2017 Dec;2017(1):168-171
    Division of Hematology, Department of Medicine, University of Ottawa, Ottawa, Ontario, Canada; and.
    Case Presentation: A 26-year-old woman experienced persistent fever (39.5°C), chills, and right-lower-quadrant tenderness 3 days after caesarean delivery. A computed tomography (CT) scan of the abdomen and pelvis with contrast revealed enlargement of her right ovarian vein with an associated intraluminal filling defect. Read More

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