EMJ Hematology最新文献

{"title":"Introducing the Concept of Patient Blood Management and Haemovigilance in Government Sector Hospitals of Karachi, Pakistan","authors":"Samra Waheed, Muhammad Khan, Shumaila Qamar, Hamid Siddiqui, Erum Rafiq","doi":"10.33590/emjhematol/yfsj1100","DOIUrl":"https://doi.org/10.33590/emjhematol/yfsj1100","url":null,"abstract":"Background and Aims: While patient blood management (PBM) and haemovigilance are different, they are both significant following evidence-based clinical transfusion. PBM is defined as the timely application of evidence-based medical and surgical concepts designed to maintain haemoglobin concentration, optimise haemostasis, and minimise blood loss in an effort to improve patient outcomes. The main aim is to manage patients such that transfusion is only used when the benefits outweigh the risks. Haemovigilance is the set of surveillance procedures covering the entire blood transfusion chain, to minimise the risk of any transfusion-related event to the donor as well as the patient.\u0000\u0000In this study, the authors audited, critiqued, and measured their own performance regarding the establishment of PBM and haemovigilance at government sector hospitals of the Regional Blood Centre Karachi, Sindh, Pakistan.\u0000\u0000Materials and Methods: A retrospective analysis of the 3.5 years since the establishment of Regional Blood Centre (RBC) Karachi and its associated hospital-based blood banks was conducted. Data were taken from Blood Bank Management Information System software and analysed on SPSS version 23 (IBM, Armonk, New York, USA) . The authors calculated the frequency of transfusion reactions and donor-related adverse events, and also calculated cross match to infusion (CT) ratio and transfusion index.\u0000\u0000Results: Initially, the cross match to transfusion ratio was 15:1, but with time and management, at the end of the third year, the team achieved a CT ratio of 1.7:1.0 for different hospitals. Transfusion index was also calculated to be 0.2 at the start of 2020 and 0.8 at the end of 2022. Similarly, no transfusion reaction was reported for the first 1.5 years since RBC’s establishment, and in the later 2 years, 59 reactions were reported. No whole blood was issued, a maximum surgical blood ordering schedule was initiated, and a restrictive transfusion strategy was applied.\u0000\u0000Conclusion: While there is still progress to be made, the authors were able to reduce wastage and subsequently suggest the idea of a restrictive transfusion strategy, so that the right blood component is given to the right patient at the right time.","PeriodicalId":509115,"journal":{"name":"EMJ Hematology","volume":"26 23","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-07-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141802819","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}

{"title":"Advanced-Stage Hodgkin Lymphoma International Prognostic Index (A-HIPI) in Turkish Patients with Classical Hodgkin Lymphoma: A Single-Center Retrospective Study","authors":"Oguzhan Koca, Berk Ozyurt, Aysenur Umar, Deniz Ozmen, T. Elverdi, A. Salihoğlu, M. Cem Ar, Z. Başlar, A. Eşkazan","doi":"10.33590/emjhematol/wzqz3587","DOIUrl":"https://doi.org/10.33590/emjhematol/wzqz3587","url":null,"abstract":"","PeriodicalId":509115,"journal":{"name":"EMJ Hematology","volume":"36 3","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-07-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141802410","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}

{"title":"A Debate on the Use of Gene Therapy in Patients with Haemophilia","authors":"Katrina Thornber","doi":"10.33590/emjhematol/qzwg2760","DOIUrl":"https://doi.org/10.33590/emjhematol/qzwg2760","url":null,"abstract":"GENE therapy is an innovative approach to treating haemophilia A and haemophilia B, with the potential to increase quality of life, promote prophylaxis, and even achieve curative factor levels in some cases. Despite early success in recent clinical trials, gene therapy for treating haemophilia is a relatively new area of research, and the long-term safety and efficacy are yet to be determined. Additionally, the current high price limits access for most patients. The suitability, safety, and accessibility of gene therapy for patients with haemophilia were discussed during a highly engaging debate session at the European Haematology Association (EHA) Congress 2024, titled ‘Haemophilia: Gene Therapy Access for Patients?’.","PeriodicalId":509115,"journal":{"name":"EMJ Hematology","volume":"35 6","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-07-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141805351","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}

{"title":"Normalisation of Haemostasis in Haemophilia A","authors":"Kristina Standeven","doi":"10.33590/emjhematol/tupo1598","DOIUrl":"https://doi.org/10.33590/emjhematol/tupo1598","url":null,"abstract":"Haemophilia A (Factor VIII [FVIII] levels ≤40 IU/dL) is a chronic condition with consequences beyond bleeding complications. Many people with haemophilia A (PwHA) experience pain, joint damage, psychosocial impacts, restrictions in daily activities, and limitations in physical activities. Cédric Hermans, Professor at the Cliniques Universitaires Saint-Luc, Brussels, Belgium, outlined how ambitious treatment goals, beyond converting severe haemophilia A into a more moderate or mild form of the condition, are required. With new treatments, it will be possible to target FVIII activity levels in the non-haemophilia range (>40 IU/dL), allowing PwHA to reach freedom from bleeds, leading to a haemophilia-free mindset, and comparable quality of life (QoL) with their peers. Maria Elisa Mancuso, Senior Haematology Consultant at IRCCS Humanitas Research Hospital, Milan, Italy, highlighted the evolution of haemophilia A treatments; she showed clinical evidence that a zero-bleed goal may require sustained FVIII activity levels >40 IU/dL for complete protection against all types of bleeds and joint damage. Rubén Berrueco, Paediatric Haematologist at the Sant Joan de Déu Barcelona Children’s Hospital, Spain, described the haemophilia paediatric patient journey, and how uncertainties related to bleeds and treatment burden pose unique challenges for children and their caregivers. He presented his perspectives on challenges with current treatments (e.g., delayed inhibitor development, subclinical bleeds, and lack of skills for intravenous administration) and the need to improve self-autonomy and decrease hospital dependency. New treatments to achieve the non-haemophilia range of FVIII could address current unmet needs. The experts discussed that treatments for many diseases (e.g., diabetes, hypertension) aim to restore normal values (blood sugar, blood pressure), which was not the case until now for haemophilia. A more patient-centred approach with treatments targeting normal values of FVIII could allow all PwHA to become mentally and physically liberated from the constraints of their condition, and to live with optimised health and well-being.","PeriodicalId":509115,"journal":{"name":"EMJ Hematology","volume":"102 3","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-07-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141802577","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}

{"title":"Filgrastim Used for Infection Prophylaxis for Moderate Neutropenia Related to Primary Myelodysplasia Prior to Elective Surgery: A Case Report","authors":"Sonya Mannala, Emmett Harrison","doi":"10.33590/emjhematol/fcvq4758","DOIUrl":"https://doi.org/10.33590/emjhematol/fcvq4758","url":null,"abstract":"Myelodysplastic syndromes are a group of disorders that affect the bone marrow, subsequently affecting the growth and relative abundance of blood-forming cells in the circulating volume. Myelodysplastic syndromes often do not cause early signs or symptoms, and can be found during routine blood tests. Granulocyte colony-stimulating factors (G-CSF) have been used in the treatment of myelodysplastic syndromes with neutropenia. Filgrastim, a G-CSF, helps increase the number of circulating neutrophils. Therefore, it has been proven to reduce patient vulnerability to infections in instances such as chemotherapy-induced neutropenia. This case report describes a 66-year-old male who presented for a pre-operative assessment before an elective left total hip arthroplasty. Routine bloodwork showed a low neutrophil count, and the surgery was cancelled due to concerns about the patient’s risk of infection. Further testing included a bone marrow aspirate and core biopsy that showed mild megaloblastic erythropoiesis and a relative increase in the proportion of myeloblasts and promyelocytes. The patient was given a working diagnosis of early myelodysplasia, and a trial of a low-dose G-CSF was started. The neutrophil count was monitored at 6–72 hours. After 72 hours of administration of filgrastim, the patient’s blood neutrophil levels had improved outside the range of neutropenia. After clearance for surgery, the patient had a successful hip arthroplasty with no post-operative infection reported. No neutropenia was noted post-surgery. This case highlights the potential of filgrastim to be used as prophylaxis before an elective surgery to improve moderate neutropenia related to primary myelodysplasia.","PeriodicalId":509115,"journal":{"name":"EMJ Hematology","volume":"36 20","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-07-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141804279","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}

{"title":"Impact of TET2 Mutations on Haematopoietic Stem Cell Resilience to Immune-Related Stress","authors":"H. Encabo, Giuseppe D’Agostino, Alessandra Ferrelli, Aneesh Sharma, Syed A. Mian, Manuel Garcia-Albornoz, Linda Ariza-McNaughton, D. Bonnet","doi":"10.33590/emjhematol/wjnj7334","DOIUrl":"https://doi.org/10.33590/emjhematol/wjnj7334","url":null,"abstract":"","PeriodicalId":509115,"journal":{"name":"EMJ Hematology","volume":"29 5","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-07-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141805027","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}

{"title":"KMT2A-MLLT3-Induced Leukaemia Changes During Ontogenic Stages","authors":"Meaad Almowaled, Lijun Liu, Karen Dunn, Elzbieta Kania, M. Aung, Gillian Horne, J. Schwaller, Karen Keeshan","doi":"10.33590/emjhematol/bnzt2573","DOIUrl":"https://doi.org/10.33590/emjhematol/bnzt2573","url":null,"abstract":"","PeriodicalId":509115,"journal":{"name":"EMJ Hematology","volume":"37 14","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-07-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141806095","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}

{"title":"Diffuse Large B Cell Lymphoma of Spleen: An Important Differential of a Nodular Splenomegaly: A Case Report","authors":"V. Punia, Aditya Chakravorty, Naman Bansal, AK Mandal, Shaavi Mittal, Akash Bharti","doi":"10.33590/emjhematol/hhiy2016","DOIUrl":"https://doi.org/10.33590/emjhematol/hhiy2016","url":null,"abstract":"Diffuse large B cell lymphoma (DLBCL) is the most common histological subtype of non-Hodgkin’s lymphoma. However, splenic DLBCL is a relatively uncommon form of non-Hodgkin’s lymphoma.1 In this case report, the authors present a 38-year-old male who was admitted to the hospital with a complaint of abdominal distension, left-sided abdominal pain, loss of weight, and loss of appetite for 2 months. The basic workup of this patient was suggestive of microcytic anaemia with a raised total lymphocyte count, platelet count, and erythrocyte sedimentation rate, while a nodular spleen with altered splenic echotexture was revealed on ultrasonography. Splenic malignancy was suspected and contrast-enhanced CT of the abdomen was planned, which gave an impression of an extra splenic mass lesion causing impingement on the spleen with continuity to bowel loops and thickening of the fascia, raising the possibility of a gastrointestinal stromal tumour; however, the presence of large conglomerated necrosed lymph nodes in the abdominal cavity pointed the diagnosis towards a splenic lymphoma. The splenectomy specimen had multiple nodular deposits and immunohistochemistry studies finally provided a clear-cut diagnosis of DLBCL–mucosa-associated lymphoid tissue lymphoma.","PeriodicalId":509115,"journal":{"name":"EMJ Hematology","volume":"50 10","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-07-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141803835","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}

{"title":"Therapeutic Approaches for Platelet Disorders","authors":"European Medical Journal","doi":"10.33590/emjhematol/fjae7013","DOIUrl":"https://doi.org/10.33590/emjhematol/fjae7013","url":null,"abstract":"","PeriodicalId":509115,"journal":{"name":"EMJ Hematology","volume":"58 12","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-07-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141805631","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}

{"title":"Five-year Outcome of CD19 Combined With CD22 CAR-T Cell Therapy in Patients With B-ALL Relapsed After Allo-transplantation","authors":"Shuangyou Liu, L. An, Zhichao Yin, Yue-hui Lin, Zhuojun Lin, B. Deng, Xinjian Yu, Q. Zheng, Defeng Zhao, Tong Wu, Alex H Chang, Chunrong Tong","doi":"10.33590/emjhematol/bofe4319","DOIUrl":"https://doi.org/10.33590/emjhematol/bofe4319","url":null,"abstract":"","PeriodicalId":509115,"journal":{"name":"EMJ Hematology","volume":"67 4","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-07-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141802374","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}