EMJ Hematology最新文献_第3页

Extension Treatment: Preventing Recurrence of Venous Thromboembolism 延伸治疗:预防静脉血栓栓塞复发

EMJ Hematology Pub Date : 2023-04-13 DOI: 10.33590/emjhematol/10306891

David J Sutton, S. Rhodes

引用次数: 0

Zamtocabtagene Autoleucel (MB-CART2019.1): An Investigational CAR-T Cell Product with Tandem Targeting of CD19 and CD20 as a Potential Treatment Option for Patients with Relapsed/ Refractory B Cell Non-Hodgkin Lymphoma Zamtocabtagene autoleuel (MB-CART2019.1):一种靶向CD19和CD20的CAR-T细胞产品，作为复发/难治性B细胞非霍奇金淋巴瘤患者的潜在治疗选择

EMJ Hematology Pub Date : 2022-07-21 DOI: 10.33590/emjhematol/22c0248

Jennifer Taylor

引用次数: 0

Von Willebrand Disease-Associated Angiodysplasia: Presentation of a Paediatric Case 血管性血友病相关血管发育不全:一例儿科病例报告

EMJ Hematology Pub Date : 2022-07-21 DOI: 10.33590/emjhematol/22-00027

S. Aggoune, Nacer Djidjli, H. Maouche

引用次数: 0

Social Impact and Quality of Life of Patients with β-Thalassaemia: A Systematic Review β-地中海贫血患者的社会影响和生活质量:一项系统综述

EMJ Hematology Pub Date : 2022-04-28 DOI: 10.33590/emjhematol/22-00041

F. Greco, F. Marino

{"title":"Social Impact and Quality of Life of Patients with β-Thalassaemia: A Systematic Review","authors":"F. Greco, F. Marino","doi":"10.33590/emjhematol/22-00041","DOIUrl":"https://doi.org/10.33590/emjhematol/22-00041","url":null,"abstract":"β-Thalassaemia (BT) is a hereditary genetic blood disease caused by a mutation in the gene that encodes the haemoglobin protein. In the most severe forms, BT forces patients to undergo frequent blood transfusions, which has a significant impact on the quality of life. Classified as rare, BT is very common in the Mediterranean area, and is also found in the Middle East, Central Asia, India, South America, and North Africa. \u0000\u0000This disease does not currently have a definitive cure, although technological progress and new gene therapies are achieving promising results. \u0000\u0000This literature review was conducted with the aim to understand how BT affects patients' lives in various social contexts in which they are involved. The authors also aimed to understand which methods are used for this assessment and the possible social actions that can help in the management of the disease. \u0000\u0000Electronic databases, including PubMed, Scopus, and Web of Science, were used to search for the articles. Related article titles were selected and reduced to the abstracts of the relevant articles, after which the selected full articles were reviewed. \u0000\u0000The reviewed articles showed consistent agreement in observing that the quality of life of patients with BT is considerably lower compared with the healthy population in terms of physical, emotional, social, and functioning at school. The negative results highlight the significance of the introduction of suitable programmes by healthcare providers, counsellors, and education authorities to provide psychosocial support, and improve academic performance. In addition, genetic counselling and intervention programmes would positively impact the lives of patients with thalassaemia.","PeriodicalId":326555,"journal":{"name":"EMJ Hematology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2022-04-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"132302226","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

IMRT/IGRT Helical Tomotherapy: A Successful Treatment of Lung Parenchyma Compression Due to Extramedullary Haematopoiesis in Β-thalassaemia – a Case Report IMRT/IGRT螺旋断层治疗:Β-thalassaemia成功治疗髓外造血所致肺实质压迫1例报告

EMJ Hematology Pub Date : 2022-03-28 DOI: 10.33590/emjhematol/21-00205

G. De Gregorio, S. D’Alessandro, G. Trapani, E. Quartuccio, A. Lo Casto, G. Evangelista

{"title":"IMRT/IGRT Helical Tomotherapy: A Successful Treatment of Lung Parenchyma Compression Due to Extramedullary Haematopoiesis in Β-thalassaemia – a Case Report","authors":"G. De Gregorio, S. D’Alessandro, G. Trapani, E. Quartuccio, A. Lo Casto, G. Evangelista","doi":"10.33590/emjhematol/21-00205","DOIUrl":"https://doi.org/10.33590/emjhematol/21-00205","url":null,"abstract":"Thalassaemia is a chronic haemolytic anaemia that is endemic in the Mediterranean Basin. Extramedullary haematopoiesis (EMH) is a natural compensatory reaction involving several organs or tissues. This report outlines a case of dyspnoea due to bilateral dorsal paravertebral EMH, which was treated successfully with helical tomotherapy, a technique that combines intensity-modulate radiation therapy with image-guided radiation therapy. By the end of the first week of treatment, an increase in the haemoglobin value (up to 7.8 g/dL) and a remarkable reduction of dyspnoea were obtained, with haemoglobin values maintained at 7.8–7.3 g/dL without further blood transfusions. At 1-year follow-up, the patient was totally asymptomatic, with a complete resolution of dyspnoea and asthenia.\u0000\u0000The therapeutic approach to EMH remains controversial because there are no pre-established protocols, with current treatments including serial blood transfusions, hydroxyurea, radiation therapy, and surgical decompression. This clinical case description reports how helical tomotherapy may be used as a valid and effective treatment for compressive atelectasis due to EMH. In fact, radiation therapy improved the general clinical condition and self-reported symptoms of the patient and reduced the size of EMH masses visible in the chest CT scan.","PeriodicalId":326555,"journal":{"name":"EMJ Hematology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2022-03-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"133645316","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

Digital Necrosis as Initial Manifestation of Multiple Myeloma: An Unusual Case Report 指状坏死为多发性骨髓瘤的初始表现:一罕见病例报告

EMJ Hematology Pub Date : 2022-02-03 DOI: 10.33590/emjhematol/21-00164

Hind Zrikem, A. Raissi, Salma Amrani Idrissi, H. Yahyaoui, Saloua Abbassi, M. Ait Ameur, M. Chakour

引用次数: 0

Atorvastan, Apsirin and Hydorxyurea for an Effective and Low-Cost Treatment in High-Risk Polycythemia Vera 阿托伐他坦、阿普sirin和羟基脲有效低成本治疗高风险真性红细胞增多症

EMJ Hematology Pub Date : 2022-01-24 DOI: 10.33590/emjhematol/21-00209

R. Amaru, Mireya Carrasco, V. Gordeuk, T. Quispe, Silvia Mancilla, D. Patón, Ariel Amaru

{"title":"Atorvastan, Apsirin and Hydorxyurea for an Effective and Low-Cost Treatment in High-Risk Polycythemia Vera","authors":"R. Amaru, Mireya Carrasco, V. Gordeuk, T. Quispe, Silvia Mancilla, D. Patón, Ariel Amaru","doi":"10.33590/emjhematol/21-00209","DOIUrl":"https://doi.org/10.33590/emjhematol/21-00209","url":null,"abstract":"Introduction: Polycythemia vera (PV) treatment focuses on preventing thrombotic events and delaying transformation to myelofibrosis or leukaemia. According to risk stratification, low-risk patients require therapeutic phlebotomy combined with acetylsalicylic acid, whilst the treatment of high-risk patients with PV relies on cytoreductive therapies, employing hydroxyurea (HU), ruxolitinib, or interferons. However, in low- and middle-income countries, the availability and cost of these drugs\u0000poses a challenge in treating high-risk patients, so optimising existing resources is required.\u0000Method: A prospective longitudinal study aimed to investigate the combination of atorvastatin (ATV), aspirin, and low-dose HU as a therapeutic strategy to treat PV in high-risk patients. The study evaluated the effect of statins on erythroid colony proliferation in vitro, as well as the applicability of ATV (20 mg/day), acetylsalicylic acid (100 mg/day), and hydroxiurea (500 mg/day) in high-risk patients with PV from La Paz, Bolivia, residing at 3,600 metres above sea level.\u0000Results: Simvastatin (3.5 μm) inhibited UKE-1 cell (JAK2V617F mutated) proliferation at 33%, and burstforming unit-erythroid colonies from patients with PV at 61%. Patients receiving ATV, aspirin, and low-dose HU displayed a good response and adequate tolerance to treatment (13-years follow-up). No patients experienced myelofibrosis or transformation to leukaemia, and no severe adverse events were observed.\u0000Conclusions: This accessible, effective, and low-cost therapeutic strategy could improve adherence to treatment and the overall survival of high-risk patients with PV in resource-limited countries.","PeriodicalId":326555,"journal":{"name":"EMJ Hematology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2022-01-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"134438522","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

EHA-ESMO Guidelines for the Diagnosis, Treatment, and Follow-up of Multiple Myeloma EHA-ESMO多发性骨髓瘤的诊断、治疗和随访指南

EMJ Hematology Pub Date : 2021-07-27 DOI: 10.33590/10.33590/emjhematol/21f10727

T. Wolf

引用次数: 0

Current and Future Therapies for β-Thalassaemia: A Review Article β-地中海贫血的当前和未来治疗:综述文章

EMJ Hematology Pub Date : 2021-07-27 DOI: 10.33590/emjhematol/20-00249

L. Giannoni, E. Angelucci

{"title":"Current and Future Therapies for β-Thalassaemia: A Review Article","authors":"L. Giannoni, E. Angelucci","doi":"10.33590/emjhematol/20-00249","DOIUrl":"https://doi.org/10.33590/emjhematol/20-00249","url":null,"abstract":"This article will review recent and forthcoming advances in the treatment of thalassaemia. Prognosis of thalassaemia has dramatically improved in the last 50 years with the development of regular and safe blood transfusions and iron chelation. Almost 20 years ago, development of oral chelators, and more recently the improvement in the knowledge and understanding of iron pathophysiology, have led to optimal iron toxicity prevention and treatment. These considerable advancements in medical therapy have transformed transfusion-dependent thalassaemia from a lethal childhood disease to a chronic disease with an open prognosis, even in those individuals over 50 years of age, and with the disease being, in some instances, curable. In the 1980s, the introduction of allogeneic haematopoietic cell transplantation provided the possibility of curing the congenital disease for the first time. More recent developments include an improved understanding of erythropoiesis, which led to the development of new erythroid-stimulating factors effective in thalassaemia, an expansion of donor pull for transplantation, and the approach of the long-term promised gene therapy in clinical practice. Moreover, ongoing trials of gene editing and agents modulating iron metabolism promise new improvements. Today, patients with thalassaemia have several weapons in their therapeutic arsenal and, hopefully, will have much more to come. As usual in medical practice, new advancements provide new challenges for the medical community, and it is the duty of this community to clearly understand the benefits and challenges of any new approach in order to provide the highest clinical benefit to patients.","PeriodicalId":326555,"journal":{"name":"EMJ Hematology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2021-07-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"123969844","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

Recurrent Episodes of Angioedema as Presenting Feature of JAK2-Positive Myeloproliferative Disorder Consistent with Polycythaemia Vera 反复发作的血管性水肿是与真性红细胞增多症一致的jak2阳性骨髓增生性疾病的表现特征

EMJ Hematology Pub Date : 2021-07-27 DOI: 10.33590/emjhematol/20-00274

Laith Al-Azrai, A. Mustafa, Adi Yousef al-Wahadneh

{"title":"Recurrent Episodes of Angioedema as Presenting Feature of JAK2-Positive Myeloproliferative Disorder Consistent with Polycythaemia Vera","authors":"Laith Al-Azrai, A. Mustafa, Adi Yousef al-Wahadneh","doi":"10.33590/emjhematol/20-00274","DOIUrl":"https://doi.org/10.33590/emjhematol/20-00274","url":null,"abstract":"Polycythaemia vera (PV) is one of the chronic myeloproliferative neoplasms, which are collectively characterised by clonal proliferation of myeloid cells with variable morphologic maturity and haematopoietic efficiency. PV is distinguished clinically from other myeloproliferative neoplasms by the presence of an elevated red blood cell mass because of uncontrolled red blood cell production. This is accompanied by increased white blood cells and platelet production, which is because of abnormal clone of haematopoietic stem cells with increased sensitivity to the different growth factors for maturation. PV can present with variable symptoms because of impaired oxygen delivery caused by slugging of blood, such as headaches, dizziness, vertigo, tinnitus, visual disturbances, and angina pectoris.\u0000\u0000Some patients present with bleeding complications (1%); another 1% of patients present with thrombotic complications. It is uncommon for patients with myeloproliferative disorders to present with features of angioedema. There are many reported cases in the literature that describe the relationship between the occurrence of angioedema and lymphoproliferative diseases; however, there are no reported cases describing instances of myeloproliferative neoplasm with angioedema.\u0000\u0000In this article, the authors have studied a case of a 53-year-old male who presented with recurrent episodes of features that are suggestive of angioedema. He was diagnosed with JAK2-positive myeloproliferative disorder consistent with PV; this is the first reported case in Jordan.","PeriodicalId":326555,"journal":{"name":"EMJ Hematology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2021-07-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"122595688","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