Annals of blood最新文献_第3页

Introductory preface of blood group genotyping 血型基因分型介绍序言

Annals of blood Pub Date : 2023-12-01 DOI: 10.21037/aob-2023-02

F. B. Clausen

引用次数: 0

The importance of patient blood management for patients, providers and the public 患者血液管理对患者、医疗服务提供者和公众的重要性

Annals of blood Pub Date : 2023-12-01 DOI: 10.21037/aob-22-39

Colleen W. Gilstad, Jessica Poisson, Rounak Dubey, Sherry Shariatmadar, Melanie Jorgenson, R. Gammon

引用次数: 0

Frequencies of ABO and RhD blood groups among blood donors in Hong Kong 香港献血者的 ABO 和 RhD 血型频率

Annals of blood Pub Date : 2023-12-01 DOI: 10.21037/aob-23-6

Yun Wing Ng, Chi Yeung Tong, W. C. Tsoi, Cheuk Kwong Lee

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RH genotyping by next-generation sequencing 通过新一代测序进行 RH 基因分型

Annals of blood Pub Date : 2023-12-01 DOI: 10.21037/aob-23-10

Aditi Khandelwal, Sandra Zittermann, Thomas Sierocinski, Celina Montemayor

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Elucidation of the molecular bases of the Rh system and its contribution to transfusion and obstetric medicine—historical and current perspective: a review 阐明 Rh 系统的分子基础及其对输血和产科医学的贡献--历史和当前视角：综述

Annals of blood Pub Date : 2023-12-01 DOI: 10.21037/aob-23-4

Carlos Cotorruelo

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Atypical hemolytic uremic syndrome: genetically-based insights into pathogenesis through an analysis of the complement regulator CD46. 非典型溶血性尿毒症综合征:通过对补体调节因子CD46的分析来了解其发病机制

Annals of blood Pub Date : 2023-09-30 Epub Date: 2023-04-11 DOI: 10.21037/aob-22-40

Xiaobo Wu, M Kathryn Liszewski, Anuja Java, John P Atkinson

{"title":"Atypical hemolytic uremic syndrome: genetically-based insights into pathogenesis through an analysis of the complement regulator CD46.","authors":"Xiaobo Wu, M Kathryn Liszewski, Anuja Java, John P Atkinson","doi":"10.21037/aob-22-40","DOIUrl":"10.21037/aob-22-40","url":null,"abstract":"The complement system is a critical innate immune defense mechanism that also facilitates antigen recognition as well as antibody production through the adaptive immune response. Overall, complement activation contributes to the immune system's recognition and response to foreign pathogens and altered self. Regulating complement activation, particularly its powerful alternative pathway (AP) amplification loop, plays a key role in modulating tissue damage at sites of injury. Besides a predisposition to infections and autoimmunity (particularly systemic lupus erythematosus) in individuals deficient in activating components, variants in complement regulators are associated with multiple diseases including atypical hemolytic uremic syndrome (aHUS), paroxysmal nocturnal hemoglobinuria (PNH), and age-related macular degeneration (AMD). In particular, the pathogenesis of aHUS is commonly related to a rare heterozygous loss-of-function (LOF) mutation in the gene for complement factor H (CFH), CD46 [membrane cofactor protein (MCP)] or factor I (CFI) or a gain-of-function (GOF) secondary to a variant in factor B (CFB) or C3. The variants associated with complement regulators are the most prevalent and clearly demonstrate that cofactor activity (CA) is essential to control complement activation and thereby avoid collateral damage to normal tissues. Importantly, multiple studies have now established the therapeutic efficacy of blocking the membrane attack complex (MAC) with a humanized monoclonal antibody that targets the fifth component, C5. In this review, we primarily focus on insights derived from the assessment of rare variants in a membrane complement inhibitor CD46 in aHUS. We also discuss the putative pathological mechanisms relative to these variants of the complement system.","PeriodicalId":72211,"journal":{"name":"Annals of blood","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2023-09-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11824723/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"47694388","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Immune thrombotic thrombocytopenic purpura: pathogenesis and novel therapies: a narrative review. 免疫性血栓性血小板减少性紫癜:发病机制和新疗法:叙述性回顾

Annals of blood Pub Date : 2023-09-30 Epub Date: 2023-01-06 DOI: 10.21037/aob-22-29

Szumam Liu, X Long Zheng

{"title":"Immune thrombotic thrombocytopenic purpura: pathogenesis and novel therapies: a narrative review.","authors":"Szumam Liu, X Long Zheng","doi":"10.21037/aob-22-29","DOIUrl":"10.21037/aob-22-29","url":null,"abstract":"Background and objectives: Immune thrombotic thrombocytopenic purpura (iTTP) is a rare, but potentially fatal blood disease, resulting from autoantibodies against A Disintegrin and Metalloprotease with ThromboSpondin Type 1 Repeats, 13 (ADAMTS13). While major progress has been made in past decades concerning early diagnosis and management of iTTP, the mechanisms underlying the formation and the mechanism of action of these autoantibodies against ADMATS13 are still unknown. This review will provide a narrative review of pathogenesis and novel therapeutics of iTTP.Methods: We did PubMed literature search using a combination of thrombotic thrombocytopenic purpura and treatment or pathogenesis from 1955 to November 2022. A total of 4,767 articles with full text were found and only relevant articles in English were further reviewed and summarized.Key content and findings: We found that the primary mechanism underlying severe ADAMTS13 deficiency in patients with iTTP is autoantibody-mediated inhibition and/or accelerated clearance of ADAMTS13 metalloprotease. Other factors including allosteric regulation and post-translational modifications (i.e., glycosylation and citrullination, and arginine methylation, etc.) may affect ADAMTS13 secretion and function and also contribute to the pathogenesis of iTTP. The standard of care for iTTP today consists of therapeutic plasma exchange, anti-von Willebrand factor (vWF) caplacizumab, and immunosuppressives (e.g., corticosteroids and rituximab), known as the triple therapy, which has significantly reduced exacerbation and mortality rates.Conclusions: We hope that the information provided in the review article helps better understand the pathogenesis of iTTP, which may guide design novel and more effective therapeutics for this potentially fatal disorder.","PeriodicalId":72211,"journal":{"name":"Annals of blood","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2023-09-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11296612/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"44674704","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Animal models for thrombotic thrombocytopenic purpura: a narrative review. 血栓性血小板减少性紫癜动物模型：叙述性综述

Annals of blood Pub Date : 2023-09-30 Epub Date: 2022-11-16 DOI: 10.21037/aob-22-18

Liang Zheng, X Long Zheng

{"title":"Animal models for thrombotic thrombocytopenic purpura: a narrative review.","authors":"Liang Zheng, X Long Zheng","doi":"10.21037/aob-22-18","DOIUrl":"10.21037/aob-22-18","url":null,"abstract":"Background and objective: Thrombotic thrombocytopenic purpura (TTP) is a potentially fatal blood disorder, resulting from severe deficiency of plasma ADAMTS13 (A Disintegrin And Metalloprotease with ThromboSpondin type 1 repeats, 13) activity. ADAMTS13 is crucial for normal hemostasis through proteolytic cleavage of ultra large von Willebrand factor (VWF). Since the discovery of ADAMTS13 in 2001, several animal models for TTP have been established. In this narrative review, we summarize the creation and characterization of the established animal models for TTP to date.Methods: We performed a literature search through PubMed from 1969 to 2022 using free text: TTP and animal model. We found 67 peer-reviewed articles but only 33 articles were included for review and 34 articles that did not discuss TTP were excluded.Key content and findings: There were genetically modified or antibody-mediated TTP models being established and fully characterized in mouse, rat, baboon, and zebrafish. However, we are still in urgent need of a true autoimmune TTP animal model.Conclusions: These animal models allowed researchers to further evaluate the contribution of various potential environmental factors and/or genetic modifiers to the pathogenesis, progression, and outcome of TTP; and to help assess the efficacy and safety of novel approaches for prevention and treatment of both hereditary and acquired TTP.","PeriodicalId":72211,"journal":{"name":"Annals of blood","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2023-09-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11326488/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"46425404","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Perioperative anaemia management 围手术期贫血管理

Annals of blood Pub Date : 2023-09-01 DOI: 10.21037/aob-22-42

Saikat Mandal, Debra L. Smith, Pelletier J. Peter, Vernon J. Louw, Suhasini Sil, Ismaila N. Ibrahim, Manideepa Maji, Shoily Nath

引用次数: 1

How should caplacizumab be used for treatment of immune thrombotic thrombocytopenic purpura? 如何使用卡普珠单抗治疗免疫性血栓性血小板减少性紫癜？

Annals of blood Pub Date : 2023-06-30 Epub Date: 2022-04-01 DOI: 10.21037/aob-21-87

Lucy Zheng, X Long Zheng

引用次数: 0