EMJ Hematology最新文献

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β-Thalassaemias: Highlights from the European Hematology Association Scientific Working Group (EHA-SWG) Scientific Meeting on Anaemias: Diagnosis and Treatment in the Omics Era β-地中海贫血:来自欧洲血液学协会科学工作组(EHA-SWG)贫血科学会议的亮点:组学时代的诊断和治疗
EMJ Hematology Pub Date : 2017-09-22 DOI: 10.33590/emjhematol/10314834
Kate L. Weatherall
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引用次数: 0
Different Therapeutic Interventions and Mechanisms of Action of Antisickling Agents Currently in Use in Sickle Cell Disease Management 目前在镰状细胞病治疗中使用的抗镰状细胞药物的不同治疗干预措施和作用机制
EMJ Hematology Pub Date : 2017-08-08 DOI: 10.33590/emjhematol/10310189
N. Imaga, O. Taiwo
{"title":"Different Therapeutic Interventions and Mechanisms of Action of Antisickling Agents Currently in Use in Sickle Cell Disease Management","authors":"N. Imaga, O. Taiwo","doi":"10.33590/emjhematol/10310189","DOIUrl":"https://doi.org/10.33590/emjhematol/10310189","url":null,"abstract":"Sickle cell disease is a genetic disorder caused by sickle haemoglobin. In many forms of the disease, the red blood cells can change shape upon deoxygenation due to abnormal sickle haemoglobin polymerisation. The haemoglobin proteins stick to each other, causing the cell to have a rigid surface and sickle shape and in the process damaging the red blood cell membrane, causing the cells to become stuck in blood vessels. This deprives the downstream tissues of oxygen and causes ischaemia and infarction (which may cause organ damage), such as stroke. Incidences of the disease are found most commonly in people of African descent and less commonly in people of Mediterranean, Latino, East Indian, and Arab descent (in that order). In African countries such as Nigeria, Gabon, Ghana, and the Republic of Congo, the prevalence of the sickle cell trait is between 20% and 30%, with the disease affecting ˜2–3% of the population. Herbal formulations prepared from plants are known as phytomedicines and are effective in keeping the patient out of a crisis state and enabling them to live stable lives in society, even though the faulty S gene is not eradicated but instead managed. This review highlights some of the therapeutic options in use in the management of sickle cell disease with a view to inspiring future research on this subject.","PeriodicalId":326555,"journal":{"name":"EMJ Hematology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2017-08-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"129743581","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 2
Thrombocytopenia: A Defect in Actin Dynamics? 血小板减少症:肌动蛋白动力学缺陷?
EMJ Hematology Pub Date : 2017-08-08 DOI: 10.33590/emjhematol/10314114
A. Standing
{"title":"Thrombocytopenia: A Defect in Actin Dynamics?","authors":"A. Standing","doi":"10.33590/emjhematol/10314114","DOIUrl":"https://doi.org/10.33590/emjhematol/10314114","url":null,"abstract":"The actin cytoskeleton plays many important roles in the lifecycle of platelets, from biogenesis from megakaryocytes, to activation and clearance from the circulation. It is therefore unsurprising that mutations in genes regulating the dynamics of this cytoskeleton lead to numerous inherited thrombocytopenias. A diverse array of proteins are affected, including actin nucleators, structural proteins, myosin motors, and transcriptional regulators. This review summarises the current understanding of how genetic dysregulation of the actin cytoskeleton can contribute to the pathogenesis of thrombocytopenia.","PeriodicalId":326555,"journal":{"name":"EMJ Hematology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2017-08-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"131216499","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 3
The Role of Allograft in Acute Lymphoblastic Leukaemia, Including Alternate Donors 同种异体移植物在急性淋巴细胞白血病中的作用,包括替代供体
EMJ Hematology Pub Date : 2017-08-08 DOI: 10.33590/emjhematol/10313027
J. Cooney
{"title":"The Role of Allograft in Acute Lymphoblastic Leukaemia, Including Alternate Donors","authors":"J. Cooney","doi":"10.33590/emjhematol/10313027","DOIUrl":"https://doi.org/10.33590/emjhematol/10313027","url":null,"abstract":"The management of acute lymphoblastic leukaemia (ALL) remains challenging. The changing landscape of newer agents and combinations of chemotherapy are improving outcomes, and various conditioning regimens and possible donor sources for allogeneic transplant provide management options; allograft remains the most potent anti-leukaemia therapy available. With improvements in treatments and monitoring of disease response, allogeneic transplantation is becoming more refined as an important option for selective patients with difficult disease. Although the paediatric ALL protocols used for adolescents and young adults are now extended towards the middle-aged patients, and newer therapeutic agents may be incorporated, there is evolving data comparing short and long-term outcomes and deliverability of treatment. Reliance on registry transplant data is inadequate in guiding optimal therapy for the individual, who may have a variety of specific needs. With the limited clinical trials in this field, it is important to continue reviewing progress and outcomes with alternative stem cell sources, such as mismatched unrelated donors, haploidentical donors, and cord blood transplants, which may cure many patients, though carry risks of treatment-related mortality and morbidity. Conditioning regimens of reduced toxicity have enabled the older and higher risk patients to proceed to allograft, but it remains hazardous. It is important to understand the features of the malignant cells, response to therapies, individual patient factors, donor stem cells available, and patient’s wishes, to help craft the current management. Allogeneic transplantation remains a very important option for ALL, and patient selection and path to transplant are continuing to evolve and be guided by ongoing clinical and laboratory data, including minimal residual disease assessment.","PeriodicalId":326555,"journal":{"name":"EMJ Hematology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2017-08-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"117288866","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Double-Hit and Triple-Hit Lymphomas: New Perspectives for Their Classification 双重打击和三重打击淋巴瘤:分类的新视角
EMJ Hematology Pub Date : 2017-08-08 DOI: 10.33590/emjhematol/10313900
Cristiano Claudino Oliveira, Maria Aparecida Custódio Domingues
{"title":"Double-Hit and Triple-Hit Lymphomas: New Perspectives for Their Classification","authors":"Cristiano Claudino Oliveira, Maria Aparecida Custódio Domingues","doi":"10.33590/emjhematol/10313900","DOIUrl":"https://doi.org/10.33590/emjhematol/10313900","url":null,"abstract":"In 2016, an update on the classification of lymphoid neoplasm was published, and one of the modifications made focussed on B-cell lymphoma, unclassifiable, with features intermediate between diffuse large B-cell lymphoma and Burkitt’s lymphoma, a term which has now been abandoned. This represented a very difficult disease in the diagnostic routine of pathologists. The new proposed term is high-grade B-cell lymphoma, which includes the double-hit lymphomas. Yet, there was still confusion about the diagnostic criteria. This review discusses the changes in classification, with an emphasis on the double-hit lymphomas. Diffuse large B-cell lymphoma and Burkitt’s lymphoma are also commented on in the text. The diagnosis of double-hit lymphomas is dependent on molecular tests and it is not available throughout the world. Research identifying features that can allow patients to be specifically selected for these molecular tests is also important.","PeriodicalId":326555,"journal":{"name":"EMJ Hematology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2017-08-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"134331293","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 1
Biosimilars for Haematologic Malignancies: The Path to Sustainable Care 恶性血液病的生物仿制药:可持续护理之路
EMJ Hematology Pub Date : 2017-08-08 DOI: 10.33590/emjhematol/10314409
E. Skinner
{"title":"Biosimilars for Haematologic Malignancies: The Path to Sustainable Care","authors":"E. Skinner","doi":"10.33590/emjhematol/10314409","DOIUrl":"https://doi.org/10.33590/emjhematol/10314409","url":null,"abstract":"The main objectives of this symposium were to review the value of biosimilars in sustainable treatment for haematologic malignancies and to recognise the developmental differences between biosimilars and their reference products. The meeting also aimed to evaluate the data on monoclonal antibodies for the treatment of haematologic malignancies and the role of biosimilars to address gaps in healthcare. Dr Cornes highlighted recent innovations in cancer treatment and presented biosimilars as economic tools that can address the financial issues that hamper progress. Prof Vulto discussed the need for healthcare professionals to be well informed about the principles of biosimilarity and aware of current and emerging therapies. Prof Jurczak presented the case for rituximab (and its biosimilars) as the standard of care for first-line B cell non-Hodgkin’s lymphoma (NHL) and its potential as maintenance treatment for indolent NHL (iNHL).","PeriodicalId":326555,"journal":{"name":"EMJ Hematology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2017-08-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"116161839","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Improving Treatment Strategies for Patients with Follicular Lymphoma: How to Translate Novel Study Data into Clinical Practice 改进滤泡性淋巴瘤患者的治疗策略:如何将新的研究数据转化为临床实践
EMJ Hematology Pub Date : 2017-08-08 DOI: 10.33590/emjhematol/10311418
Blair R. Hesp
{"title":"Improving Treatment Strategies for Patients with Follicular Lymphoma: How to Translate Novel Study Data into Clinical Practice","authors":"Blair R. Hesp","doi":"10.33590/emjhematol/10311418","DOIUrl":"https://doi.org/10.33590/emjhematol/10311418","url":null,"abstract":"Prof Bruce Cheson opened the symposium by highlighting the unmet needs for patients with follicular lymphoma (FL) and the potential application of prognostic scores, imaging techniques, and genomics to stratify patients. Ms Rosmarie Pfau detailed the challenges faced by patients with FL around the world, particularly a desire for improved quality of life (QoL) and effective treatments with less toxicity. Prof Mathias Rummel discussed modern methods of assessing FL risk and predicting treatment outcomes, particularly regarding endpoint selection for clinical trials. Dr Andrew Davies presented data from the GALLIUM study, showing that obinutuzumab-chemotherapy and maintenance is superior to rituximabchemotherapy and maintenance in untreated advanced FL patients, while Prof Gilles Salles provided insight into future options being developed for patients with FL.","PeriodicalId":326555,"journal":{"name":"EMJ Hematology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2017-08-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"125230538","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Patients in Focus: What’s Relevant for Chronic Myeloid Leukaemia and Philadelphia Chromosome-Positive Acute Lymphoblastic Leukaemia? 焦点患者:慢性髓性白血病和费城染色体阳性急性淋巴细胞白血病的相关性?
EMJ Hematology Pub Date : 2017-08-08 DOI: 10.33590/emjhematol/10312026
S. Etheridge
{"title":"Patients in Focus: What’s Relevant for Chronic Myeloid Leukaemia and Philadelphia Chromosome-Positive Acute Lymphoblastic Leukaemia?","authors":"S. Etheridge","doi":"10.33590/emjhematol/10312026","DOIUrl":"https://doi.org/10.33590/emjhematol/10312026","url":null,"abstract":"This symposium was dedicated to discussing BCR-ABL-positive chronic myeloid leukaemia (CML) and Philadelphia-positive acute lymphoblastic leukaemia (Ph+ALL). Prof Baccarani opened the symposium, highlighting the recent improvements in survival in patients with BCR-ABL-positive CML and Ph+ALL. Dr de Lavallade discussed the role of mutational analyses as part of molecular monitoring, including the use of next-generation sequencing (NGS) to assess BCR-ABL mutation status and to detect low-frequency mutations. Dr Rea reviewed treatment options for CML with tyrosine kinase inhibitors (TKI) in the second and third-line treatment settings. The session concluded with Dr Martinelli presenting mutational burden in Ph+ALL patients and treatment options for these patients, in particular, with ponatinib, emphasising the importance of early treatment initiation.","PeriodicalId":326555,"journal":{"name":"EMJ Hematology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2017-08-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"115472899","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
The Role and Regulation of Quiescence in Acute Lymphoblastic Leukaemia 静止在急性淋巴细胞白血病中的作用和调控
EMJ Hematology Pub Date : 2017-08-08 DOI: 10.33590/emjhematol/10311523
Robin L Williams, Peter M. Gordon
{"title":"The Role and Regulation of Quiescence in Acute Lymphoblastic Leukaemia","authors":"Robin L Williams, Peter M. Gordon","doi":"10.33590/emjhematol/10311523","DOIUrl":"https://doi.org/10.33590/emjhematol/10311523","url":null,"abstract":"There are ˜3,000 children, as well an additional ˜7,000 adults, diagnosed with acute lymphoblastic leukaemia (ALL) each year in the USA. This makes ALL the most common cancer diagnosed in children. It represents ˜25% of paediatric cancer diagnoses. With current therapy, most patients achieve a complete remission and many are cured. However, the prognosis remains quite poor for the ˜15–20% of children who suffer a relapse of their ALL. Improved outcomes for these relapsed patients will require either more efficacious salvage therapies or improved initial therapy that prevents ALL relapse. Thus, understanding the mechanisms by which a small population of leukaemia cells can escape therapy and contribute to relapse often months or years later is critical for improving ALL outcomes. Herein, we will review emerging clinical and laboratory research that suggest quiescence, or dormancy, is an important cellular mechanism that enhances ALL chemo-resistance and persistence, and ultimately contributes to disease relapse. Furthermore, the mechanisms that regulate this balance between leukaemia quiescence and proliferation are beginning to be elucidated and will provide new knowledge about leukaemia biology. Finally, these observations support the need for and feasibility of therapeutically targeting these quiescent, chemo-resistant ALL cells by either exploiting metabolic or signalling pathway vulnerabilities unique to quiescent cells, or by causing the release of ALL cells from the protective niche(s) that triggers and maintains ALL quiescence.","PeriodicalId":326555,"journal":{"name":"EMJ Hematology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2017-08-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"122187818","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Shifting Treatment Paradigms in Non-Hodgkin Lymphomas 非霍奇金淋巴瘤治疗模式的转变
EMJ Hematology Pub Date : 2017-08-08 DOI: 10.33590/emjhematol/10310138
Nicole Rossides
{"title":"Shifting Treatment Paradigms in Non-Hodgkin Lymphomas","authors":"Nicole Rossides","doi":"10.33590/emjhematol/10310138","DOIUrl":"https://doi.org/10.33590/emjhematol/10310138","url":null,"abstract":"Despite significant therapeutic advances in the treatment of patients with non-Hodgkin lymphoma (NHL), a significant proportion experience relapse or progression following standard immunochemotherapy (ICT). The introduction of novel targeted immunotherapy agents has potentially ushered in a new era in the management of NHL. Emerging approaches to treatment, including chemo-free regimens, targeted therapies, and immunotherapy for follicular lymphoma (FL), mantle cell lymphoma (MCL), and diffuse large B-cell lymphomas (DLBCL), have become increasingly important. Furthermore, genomic tools and biomarkers support subtyping of lymphomas and contribute greatly to identifying patients likely to respond to therapy and predict treatment outcome, thus offering a subset-specific precision medicine approach to managing NHL to both prevent and treat relapse. The latest development in the management of NHL is the use of checkpoint inhibitors to prevent cell–cell communication and tumour growth. Despite limited evidence to date, checkpoint inhibitors in combination with existing ICT may fundamentally shift the NHL treatment algorithm towards personalised immunotherapy.","PeriodicalId":326555,"journal":{"name":"EMJ Hematology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2017-08-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"134372030","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
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