• 主题
高级搜索  >
历史搜索 清空历史

您现在的位置：首页>美国卫生研究院文献>Hematology: the American Society of Hematology Education Program

期刊信息

  • 期刊名称：

    -

  • 刊频： Annual
  • NLM标题： Hematology Am Soc Hematol Educ Program
  • iso缩写： -
  • ISSN： -

年度选择

更多>>

  • 排序：
  • 显示：
  • 每页：
全选（0）
<10/14>
共275条结果
  • 1.

    【2hr】Autoimmune and other acquired neutropenias

    包量

    机译 自身免疫和其他获得性中性粒细胞减少
    • 作者：Peter E. Newburger
    • 刊名：Hematology: the American Society of Hematology Education Program
    • 2016年第1期
    摘要：This educational review addresses the diagnostic evaluation of patients for autoimmune and other forms of acquired neutropenia, including the futility of deconstructing the overlap of chronic “autoimmune,” “benign,” and “idiopathic” categories. Isolated neutropenias caused by infection, drugs, and immunologic disorders are also addressed. Discussion of management options emphasizes a conservative approach, with largely supportive care for these mostly benign and self-limited disorders.
  • 2.

    【2hr】Primary immune deficiencies with defects in neutrophil function

    包量

    机译 具有中性粒细胞功能缺陷的原发性免疫缺陷
    • 作者：Mary C. Dinauer
    • 刊名：Hematology: the American Society of Hematology Education Program
    • 2016年第1期
    摘要：Immune deficiencies resulting from inherited defects in neutrophil function have revealed important features of the innate immune response. Although sharing an increased susceptibility to bacterial and fungal infections, these disorders each have distinctive features in their clinical manifestations and characteristic microbial pathogens. This review provides an update on several genetic disorders with impaired neutrophil function, their pathogenesis, and treatment strategies. These include chronic granulomatous disease, which results from inactivating mutations in the superoxide-generating nicotinamide dinucleotide phosphate oxidase. Superoxide-derived oxidants play an important role in the control of certain bacterial and fungal species, and also contribute to the regulation of inflammation. Also briefly summarized are updates on leukocyte adhesion deficiency, including the severe periodontal disease characteristic of this disorder, and a new immune deficiency associated with defects in caspase recruitment domain–containing protein 9, an adaptor protein that regulates signaling in neutrophils and other myeloid cells, leading to invasive fungal disease.
  • 3.

    【2hr】Pure red cell aplasia

    包量

    机译 纯红细胞发育不良
    • 作者：Robert T. Means, Jr
    • 刊名：Hematology: the American Society of Hematology Education Program
    • 2016年第1期
    摘要：Pure red cell aplasia (PRCA) is a syndrome defined by a normocytic normochromic anemia with severe reticulocytopenia and marked reduction or absence of erythroid precursors from the bone marrow. Diamond-Blackfan anemia is a congenital form of PRCA. Acquired PRCA may be either a primary disorder or secondary to some other disorder or agent. Primary acquired PRCA is an autoimmune disorder that is frequently antibody-mediated. Myelodysplastic syndromes may also present with the morphologic appearance of PRCA. Secondary acquired PRCA may be associated with collagen vascular/autoimmune disorders such as systemic lupus erythematosus; lymphoproliferative disorders such as chronic lymphocytic leukemia or large granular lymphocyte leukemia; infections, particularly B19 parvovirus; thymoma and other solid tumors; or a variety of other disorders, drugs, or toxic agents. The therapeutic approach to PRCA typically involves immunosuppression, but specific pathogenic subtypes are associated with specific therapeutic approaches. Cyclosporine A, with or without concurrent corticosteroids, appears to be the single most effective immunosuppressive agent.
  • 4.

    【2hr】Single-dose intravenous iron for iron deficiency: a new paradigm

    包量

    机译 单剂量静脉补铁治疗铁缺乏症的新范例
    摘要：Iron-deficiency anemia is the most common hematologic problem in the world. Although oral iron is often viewed as front-line therapy, extensive published evidence has accumulated that IV iron is superior, in both efficacy and safety, to oral iron in many clinical situations and should be introduced much sooner in the treatment paradigm of iron-deficient patients. In this chapter, we will review the formulations of IV iron that allow total complete replacement doses in 1 or 2 sessions including practical tips for administration. We realize safety concerns abound and therefore will analyze evidence based overstated concerns regarding serious adverse events highlighting unnecessary interventions for minor, self-limiting infusion reactions, which infrequently occur with intravenous iron administration. Recent data for the use of IV iron in a variety of clinic situations will be reviewed including women with heavy uterine bleeding, pregnancy, bariatric surgery, inflammatory bowel disease, and restless legs syndrome. Briefly discussed is the new frontier of IV iron’s use in the prevention of acute (high altitude) mountain sickness. It is clear that in many clinical situations IV iron is a new and improved standard of care offering advantages over oral iron in efficacy, toxicity, and convenience to patients and health care providers.
  • 5.

    【2hr】New challenges in evaluating anemia in older persons in the era of molecular testing

    包量

    机译 分子测试时代评估老年人贫血的新挑战
    • 作者：David P. Steensma
    • 刊名：Hematology: the American Society of Hematology Education Program
    • 2016年第1期
    摘要：Anemia is common in older persons, and often remains unexplained despite a thorough clinical history, physical examination, and focused laboratory testing, including marrow aspiration, biopsy, and karyotyping. The advent of molecular genetic testing panels in hematology clinical practice has complicated the evaluation of older patients with unexplained anemia. While the presence of a somatic mutation provides evidence of clonal hematopoiesis and may support a diagnosis of a hematologic neoplasm such as one of the myelodysplastic syndromes (MDS), with rare exceptions, individual mutations are not strongly associated with one specific diagnosis, nor are they by themselves diagnostic of neoplasia. A clonal mutation in a patient with cytopenias and a nondiagnostic bone marrow may indicate a syndrome with a similar natural history to MDS, but at present there are no clear criteria to distinguish cytopenias coincidentally seen in association with an unrelated clonal mutation from cytopenias that are directly caused by that mutation. Ongoing and planned analyses will help define when mutation patterns alone can identify a disorder equivalent to a morphologically defined myeloid neoplasm such as MDS, further clarifying the etiology and natural history of unexplained anemia in the elderly.
  • 6.

    【2hr】Aplastic anemia and clonal evolution: germ line and somatic genetics

    包量

    机译 再生障碍性贫血和克隆进化：种系和体细胞遗传学
    • 作者：Akiko Shimamura
    • 刊名：Hematology: the American Society of Hematology Education Program
    • 2016年第1期
    摘要：Clonal progression to myelodysplastic syndrome (MDS) or acute myeloid leukemia (AML) remains a dreaded complication for a subset of patients with bone marrow failure (BMF). Recognizing risk factors for the development of MDS or AML would inform individualized treatment decisions and identify patients who may benefit from early or upfront hematopoietic stem cell transplantation. Now that next-generation DNA sequencing is available in the clinical laboratory, research has focused on the implications of germ line and somatic mutations for diagnosing and monitoring patients with BMF. Most germ line genetic BMF disorders are characterized by a high propensity to develop MDS or AML. Many affected patients lack the physical stigmata traditionally associated with the inherited marrow failure syndromes. Although any single inherited marrow failure disorder is rare, multiplexed genetic sequencing that allows simultaneous evaluation of marrow failure genes en masse demonstrated that, as a group, these inherited disorders compose a significant subset (5% to 10%) of patients with BMF. Early diagnosis of a germ line genetic marrow failure disorder allows individualized monitoring and tailored therapy. Recent studies of somatic variants in marrow failure revealed a high frequency of clonal hematopoiesis with the acquisition of mutations in genes associated with MDS or AML. Investigation of somatic mutations in marrow failure revealed important insights into the mechanisms promoting clonal disease but also raised additional questions. This review will focus on the evaluation and implications of germ line and somatic mutations for the development of clonal disorders in patients with BMF. Challenges and limitations of clinical genetic testing will be explored.
  • 7.

    【2hr】Nontransplant therapy for bone marrow failure

    包量

    机译 非移植治疗骨髓衰竭
    摘要：Nontransplant therapeutic options for acquired and constitutional aplastic anemia have significantly expanded during the last 5 years. In the future, transplant may be required less frequently. That trilineage hematologic responses could be achieved with the single agent eltrombopag in refractory aplastic anemia promotes new interest in growth factors after years of failed trials using other growth factor agents. Preliminary results adding eltrombopag to immunosuppressive therapy are promising, but long-term follow-up data evaluating clonal evolution rates are required before promoting its standard use in treatment-naive disease. Danazol, which is traditionally less preferred for treating cytopenias, is capable of preventing telomere attrition associated with hematologic responses in constitutional bone marrow failure resulting from telomere disease.
  • 8.

    【2hr】Transplantation for bone marrow failure: current issues

    包量

    机译 骨髓衰竭移植：当前问题
    • 作者：Régis Peffault de Latour
    • 刊名：Hematology: the American Society of Hematology Education Program
    • 2016年第1期
    摘要：The preferred treatment of idiopathic aplastic anemia (AA) is allogeneic hematopoietic stem cell transplantation (HSCT) from a human leukocyte antigen (HLA)–identical sibling donor. Transplantation from a well-matched unrelated donor (MUD) may be considered for patients without a sibling donor after failure of immunosuppressive therapy, as may alternative transplantation (mismatched, cord blood or haplo-identical HSCT) for patients without a MUD. HSCT may also be contemplated for congenital disorders in cases of pancytopenia or severe isolated cytopenia. Currently, HSCT aims are not only to cure patients but also to avoid long-term complications, notably chronic graft-versus-host disease (GVHD), essential for a good quality of life long term. This paper summarizes recent advances in HSCT for idiopathic and inherited AA disorders. The effect of age on current transplantation outcomes, the role of transplantation in paroxysmal nocturnal hemoglobinuria, and the prevention of GVHD are also discussed. Emerging strategies regarding the role of up-front unrelated donor and alternative donor HSCT in idiopathic AA, along with advances in the treatment of clonal evolution in Fanconi anemia, are also examined.
  • 9.

    【2hr】Biologic vs physiologic age in the transplant candidate

    包量

    机译 移植候选人的生物年龄与生理年龄
    • 作者：Andrew S. Artz
    • 刊名：Hematology: the American Society of Hematology Education Program
    • 2016年第1期
    摘要：Hematopoietic cell transplantation (HCT) remains a cornerstone of treatment of many hematologic malignancies but transplant-associated morbidity and mortality limit application to older patients. Biologic or chronologic age barriers to HCT have fallen, because patients in their 8th decade of life comprise the group with the greatest rise in transplant use over the past decade. Evaluating physiologic age or general health in older transplant candidates requires a systematic approach inclusive of functional and comorbidity assessment, which typically is accomplished through geriatric assessment (GA). GA incorporates measures of comorbidity, function, nutrition, social support, and other health-related domains to better describe physiologic age. Older allogeneic transplant patients have a surprisingly high prevalence of vulnerabilities by GA prior to transplant, and significant comorbidity or functional limitations heighten the risks of transplant-related mortality. Ultimately, incorporation of physiologic age can improve estimates of nondisease life expectancy, prognostic survival after HCT, and inform HCT candidacy. Future research on the optimal tools to characterize physiologic age and appropriate interventions in the context of transplant are needed.
  • 10.

    【2hr】Can we make a better match or mismatch with KIR genotyping?

    包量

    机译 我们能否通过KIR基因分型更好地匹配或错配？
    摘要：Natural killer (NK) cell function is regulated by a fine balance between numerous activating and inhibitory receptors, of which killer-cell immunoglobulin-like receptors (KIRs) are among the most polymorphic and comprehensively studied. KIRs allow NK cells to recognize downregulation or the absence of HLA class I molecules on target cells (known as missing-self), a phenomenon that is commonly observed in virally infected cells or cancer cells. Because KIR and HLA genes are located on different chromosomes, in an allogeneic environment such as after hematopoietic stem cell transplantation, donor NK cells that express an inhibitory KIR for an HLA class I molecule that is absent on recipient targets (KIR/KIR-ligand mismatch), can recognize and react to this missing self and mediate cytotoxicity. Accumulating data indicate that epistatic interactions between KIR and HLA influence outcomes in several clinical conditions. Herein, we discuss the genetic and functional features of KIR/KIR-ligand interactions in hematopoietic stem cell transplantation and how these data can guide donor selection. We will also review clinical studies of adoptive NK cell therapy in leukemia and emerging data on the use of genetically modified NK cells that could broaden the scope of cancer immunotherapy.
  • 11.

    【2hr】Role of the intestinal mucosa in acute gastrointestinal GVHD

    包量

    机译 肠粘膜在急性胃肠道GVHD中的作用
    摘要：Intestinal graft-versus-host disease (GVHD) remains a significant obstacle to the success of allogeneic hematopoietic cell transplantation. The intestinal mucosa comprises the inner lining of the intestinal tract and maintains close proximity with commensal microbes that reside within the intestinal lumen. Recent advances have significantly improved our understanding of the interactions between the intestinal mucosa and the enteric microbiota. Changes in host mucosal tissue and commensals posttransplant have been actively investigated, and provocative insights into mucosal immunity and the enteric microbiota are now being translated into clinical trials of novel approaches for preventing and treating acute GVHD. In this review, we summarize recent findings related to aspects of the intestinal mucosa during acute GVHD.
  • 12.

    【2hr】Sequencing of chronic lymphocytic leukemia therapies

    包量

    机译 慢性淋巴细胞白血病治疗的测序
    • 作者：Jacqueline C. Barrientos
    • 刊名：Hematology: the American Society of Hematology Education Program
    • 2016年第1期
    摘要：It is an unprecedented time for the treatment of patients with chronic lymphocytic leukemia (CLL) with the recent approval of several targeted agents for use in frontline, relapsed, refractory, and high-risk disease. Traditionally, frontline management of CLL has been a combination of chemotherapy (fludarabine, cyclophosphamide, bendamustine, or chlorambucil) with an anti-CD20 monoclonal antibody (rituximab, ofatumumab, obinutuzumab). The current landscape is rapidly evolving with the advent of therapies that demonstrate selective inhibition of important pathways necessary for CLL proliferation and survival. Despite considerable progress, much is still unknown and optimal treatment selection and sequence is still debatable. None of the new agents have been compared against each other and the impact of adding an additional agent to monotherapy is not yet fully elucidated. In routine clinical practice, the choice of therapy is based on nonrandomized comparisons, presence of comorbidities, and toxicity considerations. These recently approved drugs (ibrutinib, idelalisib, and venetoclax) are reporting excellent outcomes, including patients with high-risk disease such as 17p deletion (17p−) or TP53 mutations (TP53mut). Ibrutinib and venetoclax have been approved for use in 17p− patients (frontline and relapsed, respectively). Ibrutinib is currently moving into the frontline space given recent regulatory approvals. This review will summarize and interpret the limited therapeutic sequencing data available, highlighting the need for additional studies to optimize combination strategies and treatments after failure or discontinuation of these novel agents.
  • 13.

    【2hr】Novel agents in chronic lymphocytic leukemia

    包量

    机译 慢性淋巴细胞白血病的新型药物
    摘要：The advent of novel small-molecule inhibitors has transformed the treatment approaches for patients with chronic lymphocytic leukemia (CLL). These therapies are becoming increasingly used in patients with relapsed disease, patients with 17p deletion, and, as of recently, also in the frontline setting for previously untreated patients with CLL. Moreover, many of these are oral therapies that are significantly less myelosuppressive than chemoimmunotherapy. However, these agents have their own set of unique toxicities with which providers must gain familiarity. There is also ongoing development of second-generation agents which have the promise of less toxicity than the US Food and Drug Administration (FDA)–approved compounds. In addition, immunotherapy and the role of the microenvironment are becoming increasingly important and have therapeutic implications in the treatment of patients with CLL. Ultimately, investigators need to evaluate how to position these and other new exciting therapies and decide on the ultimate role for chemoimmunotherapy in modern times.
  • 14.

    【2hr】First-line therapy for young patients with CLL

    包量

    机译 青年CLL患者的一线治疗
    • 作者：Nitin JainSusan O’Brien
    • 刊名：Hematology: the American Society of Hematology Education Program
    • 2016年第1期
    摘要：A 61-year-old man with a history of chronic lymphocytic leukemia (CLL) presents with complaints of worsening fatigue and night sweats. He was diagnosed with CLL 3 years ago on routine blood count testing. He has no major medical comorbidities. On examination, he has several 2- to 3-cm lymph nodes in the cervical and axillary area. Spleen is palpable 5 cm below the costal margin. Blood counts show lymphocytosis with thrombocytopenia and anemia. Prognostic markers include deletion 13q by fluorescence in situ hybridization analysis and mutated IGHV. You are asked by the hematology fellow you are supervising about the best treatment of this patient.
  • 15.

    【2hr】Prognostication of chronic lymphocytic leukemia in the era of new agents

    包量

    机译 新药时代下慢性淋巴细胞白血病的预后
    摘要：The prognosis of chronic lymphocytic leukemia (CLL) is very heterogeneous. Therefore, a plethora of prognostic factors has been identified to allow a better prediction of the individual prognosis of a given patient. The clinical staging systems by Rai and Binet have been the backbone of clinical management for several decades. The advent of genetic and biochemical markers, as well as next-generation sequencing has provided several markers that can predict the prognosis of patients with CLL. Using this knowledge, several scores have been created to improve predicting overall survival and/or treatment-free survival. These prognostic scores were developed in the era of chemotherpay/chemoimmunotherapy. Therefore, they now need to be tested with novel agents. However, despite tremendously improved therapeutic options, CLL patients with TP53 dysfunction or a complex karyotype remain at very high risk and seem to have a shorter (treatment-free) survival. The recently published international prognostic index (CLL IPI) incorporates most of these factors and provides a tool to analyze outcome in the modern era of targeted therapies.
  • 16.

    【2hr】Molecular monitoring in chronic myeloid leukemia—how low can you go?

    包量

    机译 慢性粒细胞白血病的分子监测-您能降低多少？
    • 作者：Susan Branford
    • 刊名：Hematology: the American Society of Hematology Education Program
    • 2016年第1期
    摘要：Molecular monitoring of BCR-ABL1 transcripts for patients with chronic myeloid leukemia (CML) is now used to assess response to tyrosine kinase inhibitors (TKIs), including treatment failure that mandates a change of therapy. Therefore, many centers have adopted the molecular technique for measuring BCR-ABL1 and rely on conversion of values to the international reporting scale for appropriate clinical interpretation. However, the technique has a degree of inherent variability despite standardized procedures, which means care should be taken by the clinician when assessing response based on BCR-ABL1 cutoff limits. The last few years have witnessed the emergence of a new molecular response target, which is the achievement and maintenance of a deep molecular response. The ability to achieve treatment-free remission for some patients has shifted the relevant boundary for molecular response. However, the definitive safe BCR-ABL1 transcript level and length of the maintenance phase after which treatment cessation can be attempted has not yet been determined. For patients with TKI resistance, BCR-ABL1 kinase domain mutation analysis remains an essential assessment to guide therapy. Furthermore, low-level mutation detection is clinically relevant for response prediction to subsequent TKI therapy for some patients. Multiple low-level mutations may be a biomarker of a clonally diverse disease with the propensity for resistance evolution. Overall, molecular monitoring, including low-level monitoring is a fundamental component of management for patients with CML.
  • 17.

    【2hr】Chronic myeloid leukemia: sequencing of TKI therapies

    包量

    机译 慢性粒细胞白血病：TKI治疗的测序
    摘要：Multiple tyrosine kinase inhibitors (TKIs) are available for managing patients with chronic myeloid leukemia. Although most patients have a favorable outcome with their initial therapy, whether imatinib or a second-generation TKI was used, some will require subsequent use of one or more different TKIs. Such sequencing might be indicated in a reactive way (ie, for patients who have experienced resistance or intolerance to their initial therapy) or in a proactive way (ie, for patients with a somewhat favorable outcome who have not reached an “optimal” outcome). Sequencing of TKIs has become standard practice, and the proper use of sequenced TKIs is likely to optimize outcomes and resource utilization.
  • 18.

    【2hr】The value of quality of life assessment in chronic myeloid leukemia patients receiving tyrosine kinase inhibitors

    包量

    机译 生活质量评估对接受酪氨酸激酶抑制剂的慢性粒细胞白血病患者的价值
    摘要：The development of the oral tyrosine kinase inhibitors (TKIs) to treat chronic myeloid leukemia (CML) is one of the great triumphs of cancer research. Although the efficacy of TKIs has dramatically improved the disease-specific overall survival rate, the prevalence of CML is increasing worldwide. Currently, CML patients receive prolonged (even lifelong) treatment, and over the last decade, clinical decision making has become challenging. Therefore, consideration of the effects of TKI therapies on patients’ quality of life (QoL) and symptom burden (ie, patient-reported outcomes [PROs]) is now critical to more robustly inform patient care and improve health care quality. Over the last 5 years, a number of studies have generated valuable PRO data, for example, on long-term QoL effects of imatinib therapy or symptom burden of patients switching from imatinib to second-generation TKIs. PRO findings are important, as they provide a unique patient perspective on the burden of the disease and treatments effects. We will review main evidence-based data on the use of PROs in clinical research and highlight the importance of methodological rigor of PRO assessment. Also, we will describe the potential value of using PRO assessment in routine clinical practice, for example, to facilitate timely management of side effects. Areas for future research will also be discussed.
  • 19.

    【2hr】Thrombosis in the setting of obesity or inflammatory bowel disease

    包量

    机译 肥胖或炎症性肠病的血栓形成
    • 作者：Steven R. Lentz
    • 刊名：Hematology: the American Society of Hematology Education Program
    • 2016年第1期
    摘要：Obesity and inflammatory bowel disease (IBD) are systemic inflammatory disorders that predispose to arterial and venous thrombosis through similar prothrombotic mechanisms. Obesity and IBD are chronic risk factors that lead to a persistently elevated risk of thrombosis, although the thrombotic risk with IBD appears to wax and wane with disease severity. Because of the lack of high-quality evidence to guide management decisions, approaches to the prevention and treatment of thrombosis in patients with obesity or IBD are based on extrapolation from general guidelines for antithrombotic therapy. Obesity alters the pharmacokinetics of some anticoagulant drugs, and IBD patients present the added management challenge of having a high risk of gastrointestinal bleeding while taking anticoagulants. An extended duration of anticoagulant therapy is often recommended for obese or IBD patients with unprovoked venous thromboembolism unless there is a high risk of bleeding, although more data and better biomarkers are needed to determine whether anticoagulation can be safely stopped in a subset of IBD patients during remission of active disease. Most patients with obesity or IBD require thromboprophylaxis in conjunction with hospitalization or surgery, with adjustment of anticoagulant dosing in patients with severe obesity.
  • 20.

    【2hr】Thrombosis and anticoagulation in the setting of renal or liver disease

    包量

    机译 肾脏或肝脏疾病时的血栓形成和抗凝作用
    摘要：Thrombosis and bleeding are among the most common causes of morbidity and mortality in patients with renal disease or liver disease. The pathophysiology underlying the increased risk for venous thromboembolism and bleeding in these 2 populations is distinct, as are considerations for anticoagulation. Anticoagulation in patients with kidney or liver disease increases the risk of bleeding; this risk is correlated with the degree of impairment of anticoagulant elimination by the kidneys and/or liver. Despite being in the same pharmacologic category, anticoagulant agents may have varied degrees of renal and liver metabolism. Therefore, specific anticoagulants may require dose reductions or be contraindicated in renal impairment and liver disease, whereas other drugs in the same class may not be subject to such restrictions. To minimize the risk of bleeding, while ensuring an adequate therapeutic effect, both appropriate anticoagulant drug choices and dose reductions are necessary. Renal and hepatic function may fluctuate, further complicating anticoagulation in these high-risk patient groups.
题名 作者 来源 发表时间

客服邮箱：kefu@zhangqiaokeyan.com

京公网安备：11010802029741号 ICP备案号：京ICP备15016152号-6 六维联合信息科技 (北京) 有限公司©版权所有

  • 客服微信

  • 服务号