EMJ Hematology最新文献_第5页

Pregnancy-Related Thromboembolismin Sickle Cell Disease 妊娠相关血栓栓塞性镰状细胞病

EMJ Hematology Pub Date : 2020-07-30 DOI: 10.33590/emjhematol/20-00037

Salma M. AlDallal

{"title":"Pregnancy-Related Thromboembolismin Sickle Cell Disease","authors":"Salma M. AlDallal","doi":"10.33590/emjhematol/20-00037","DOIUrl":"https://doi.org/10.33590/emjhematol/20-00037","url":null,"abstract":"Haematological disorders are predominant in the tropical and subtropical countries where major problems of sickle-cell disease (SCD) and thalassaemias are often recorded. However, reports of these conditions have increased in the Western hemisphere more recently. Genetic counselling, early detection of the disease condition, and determining an appropriate treatment regimen remains the solution. Most molecular types of SCD have been determined and the pathological impact of individual types along with the degree of severity is known to clinical investigators and physicians. There is, however, a significant need for a proper counselling system for the clinical diagnosis in most countries. Lack of funding, trained personnel, relevant physicians, instruments, and laboratories are the challenges to overcome. Pregnancy-associated SCD and thromboembolism require special mention due to their mortality rate, complexity of treatment, and care necessities. This review considers some of the most important aspects of pregnancy-associated SCD and thromboembolism, shedding light on the present understanding of the disease condition, pathology, clinical issues, the association with venous thromboembolism, recent treatment measures, and clinical and social management of pregnant women and fetuses for patients with SCD. Integrated social and clinical care along with extensive timely medical and clinical counselling for patients can improve the present situation which is growing in different countries. To save future generations and pregnant mothers from the haematological disorders that could be either prevented or treated, essential genetic screening or counselling should be made a priority by governments. In addition, social education and campaigns related to the disease condition can help to improve the situation.","PeriodicalId":326555,"journal":{"name":"EMJ Hematology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2020-07-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"127345145","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

Pernicious Anaemia: Mechanisms, Diagnosis, and Management 恶性贫血:机制、诊断和管理

EMJ Hematology Pub Date : 2020-01-24 DOI: 10.33590/emjhematolu/19-00187

W. Ammouri, H. Harmouche, H. Khibri, S. Benkirane, Masrar Azlarab, Z. Tazi, M. Maamar, M. Adnaoui

{"title":"Pernicious Anaemia: Mechanisms, Diagnosis, and Management","authors":"W. Ammouri, H. Harmouche, H. Khibri, S. Benkirane, Masrar Azlarab, Z. Tazi, M. Maamar, M. Adnaoui","doi":"10.33590/emjhematolu/19-00187","DOIUrl":"https://doi.org/10.33590/emjhematolu/19-00187","url":null,"abstract":"Pernicious anaemia (PA) is an autoimmune disease of multifactorial aetiology involving environmental and immunological factors. It is the most common cause of cobalamin deficiency anaemia worldwide. The disease is a macrocytic anaemia caused by a vitamin B12 deficiency, which, in turn, is the result of intrinsic factor deficiency, a protein that binds avidly to dietary vitamin B12 and promotes its transport to the terminal ileum for absorption. Despite the advances in understanding the pathogenesis and molecular biology, diagnosis of PA is still challenging for clinicians because of its complexity, diverse clinical presentations, and the limitations of the available diagnostic tools for the evaluation of cobalamin status and the presence of chronic autoimmune atrophic gastritis. Asymptomatic autoimmune gastritis, a chronic inﬂammatory disease of the gastric mucosa, precedes the onset of corpus atrophy by 10–20 years. Diagnostic dilemmas could occur when patients with PA present with spuriously normal or high cobalamin levels, normocytic or microcytic anaemia, nonanaemic macrocytosis, autoimmune haemolytic anaemia, pseudo-thrombotic microangiopathy, hyperhomocysteinemia-associated thromboembolism, pseudoleukemia, bone marrow failure, and neurologic manifestations without anaemia or macrocytosis. Other autoimmune disorders, especially thyroid disease, Type 1 diabetes mellitus, and vitiligo, are also commonly associated with PA. The present review focusses on novel aspects regarding the pathogenesis, clinical presentation, and the diagnostic approach of PA; the true usefulness of serum vitamin B12 levels; and the risk of adenocarcinoma and gastric carcinoids as well as their treatment and monitoring strategies.","PeriodicalId":326555,"journal":{"name":"EMJ Hematology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2020-01-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"124035072","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

Acute Chest Syndrome in Children with Sickle Cell Disease: Current Perspectives on Pathogenesis and Treatment 镰状细胞病儿童急性胸综合征:发病机制和治疗的最新观点

EMJ Hematology Pub Date : 2020-01-24 DOI: 10.33590/emjhematolus/19-00178

S. Uwaezuoke

{"title":"Acute Chest Syndrome in Children with Sickle Cell Disease: Current Perspectives on Pathogenesis and Treatment","authors":"S. Uwaezuoke","doi":"10.33590/emjhematolus/19-00178","DOIUrl":"https://doi.org/10.33590/emjhematolus/19-00178","url":null,"abstract":"Acute chest syndrome (ACS) is the most prominent cause of mortality in children with sickle cell disease. Its cause was initially not clearly understood, but there are now established concepts regarding its aetiopathogenesis. This narrative review discusses the current perspectives on sickle cell disease pathogenesis and treatment. The PubMed database was searched for articles that met the review objective. The major causative factors are pulmonary infections, pulmonary infarction, and pulmonary fat embolism from bone marrow necrosis. These factors initiate events that result in ACS, in which a vicious cycle of infarction, inflammation, and lung collapse occurs, leading to ventilation-perfusion mismatch and hypoxaemia. ACS is best managed in hospital settings because intensive care of the patient may be required. Despite its complex management, the primary treatment modalities are supportive care, transfusion therapy, and pharmacotherapy. Although the efficacy of several modalities in attenuating or preventing ACS are well established, the outcomes from instituting others are not convincing. More research is, therefore, needed to strengthen the evidence for their therapeutic efficacy.","PeriodicalId":326555,"journal":{"name":"EMJ Hematology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2020-01-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"126212363","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

Reed Sternberg-Like Cells in an Aggressive Lymphoma: Report of a Rare Case and Review of Literature 侵袭性淋巴瘤中的Reed sternberg样细胞:一例罕见病例报告及文献复习

EMJ Hematology Pub Date : 2020-01-24 DOI: 10.33590/emjhematolus/19-00186

S. Ahuja, V. Bharati, A. Gupta, P. Joshi, R. Kant, Ashutosh Kumar Singh

引用次数: 1

‘Girdle Syndrome’ Progressing to Ischaemic Colitis and Acute Intrahepatic Cholestasis in a Patient with Sickle Cell Disease: A Case Report 镰状细胞病患者的“腰带综合征”进展为缺血性结肠炎和急性肝内胆汁淤积1例报告

EMJ Hematology Pub Date : 2020-01-24 DOI: 10.33590/emjhematolus/19-00116

K. Manganas, S. Delicou, A. Xydaki, J. Koskinas

引用次数: 1

New Insights in β-Thalassaemia β-地中海贫血的新见解

EMJ Hematology Pub Date : 2019-08-01 DOI: 10.33590/emjhematol/10314844

E. Paubelle, X. Thomas

引用次数: 0

Rebooting the Myeloma Treatment Programme 重新启动骨髓瘤治疗计划

EMJ Hematology Pub Date : 2019-08-01 DOI: 10.33590/emjhematol/10311051

Alan E. Corcoran

引用次数: 0

Are Successful Pregnancies an Achievable Goal in Patients with Chronic Myeloid Leukaemia? 慢性髓性白血病患者成功怀孕是一个可实现的目标吗?

EMJ Hematology Pub Date : 2019-08-01 DOI: 10.33590/emjhematol/10310423

Z. Grudeva-Popova, E. Spasov, Vasko Graklanov, Katya N Sotirova, V. Popov, G. Balatzenko, I. Hristova

引用次数: 0

Retrospective Review of the Role of Abdominal Imaging in Evaluation of Cytopenias 腹部影像学在评估细胞减少症中的作用的回顾性研究

EMJ Hematology Pub Date : 2019-08-01 DOI: 10.33590/emjhematol/10314307

A. Yacoub, J. Mahnken, L. Holcomb

引用次数: 0

Eltrombopag-Induced Myelofibrosis in Patients with Adult Immune Thrombocytopenia: Scoping Review 成人免疫性血小板减少症患者依曲巴格诱导的骨髓纤维化:范围综述

EMJ Hematology Pub Date : 2019-08-01 DOI: 10.33590/emjhematol/10313783

Iman Saleh Moustafa, M. Badawy, S. Moustafa, Fetouh

{"title":"Eltrombopag-Induced Myelofibrosis in Patients with Adult Immune Thrombocytopenia: Scoping Review","authors":"Iman Saleh Moustafa, M. Badawy, S. Moustafa, Fetouh","doi":"10.33590/emjhematol/10313783","DOIUrl":"https://doi.org/10.33590/emjhematol/10313783","url":null,"abstract":"Immune thrombocytopenic purpura is a clinical syndrome of thrombocytopenia that manifests as a bleeding tendency, typical skin rashes, easy bruising, or extravasation of blood from the capillaries. Defects in the thrombopoietin-receptor (TPOR)/myeloproliferative leukaemia virus/JAK2 axis leads to haematological diseases such as thrombocytopenia or pancytopenia through the inhibition of the megakaryopoiesis process.\u0000\u0000Thrombopoietin-receptor agonists (TPORA), such as eltrombopag, increase platelet count by stimulating the TPOR. Bone marrow (BM) fibrosis has been reported in patients receiving TPORA. Myelofibrosis (MF) may be induced by mutations in JAK2, CALR, and MPL genes. This review gives an insight on MF as a serious side effect induced by eltrombopag.\u0000\u0000This review enriches the evidence of MF induced by eltrombopag after long-term administration ranging from 6 months to 7 years. MF is mostly spontaneous and decreases after discontinuation of medication; however, in a few cases it becomes persistent. This major issue should be treated with high concern. The authors recommend that any patient on eltrombopag treatment should be under vigilant observation and closely monitored for any sign of MF by clinical manifestation and any abnormal result from peripheral blood smear examination, and should additionally undergo BM biopsy for confirmation and detection of the severity of MF. The authors recommend discontinuing the medication if this side effect occurs. The authors also recommend to conduct larger studies for longer periods using serial BM before, and periodically after, eltrombopag treatment to evaluate the characteristics of this adverse effect.","PeriodicalId":326555,"journal":{"name":"EMJ Hematology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2019-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"114689795","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