止血的进展(第一部分):遗传性血小板疾病的改进管理:现实还是幻想?

IF 2.7 4区 医学 Q2 HEMATOLOGY
Werner Streif
{"title":"止血的进展(第一部分):遗传性血小板疾病的改进管理:现实还是幻想?","authors":"Werner Streif","doi":"10.1055/a-2031-7790","DOIUrl":null,"url":null,"abstract":"<p><p>Platelets are key drivers of hemostasis. Low platelet counts, dysfunction in platelet adhesion, and aggregation lead to increased bleeding tendency. Inherited platelet disorders (IPDs) form a highly heterogeneous group of rare diseases with variable bleeding tendency. IPDs may be associated with other signs and symptoms often referred to as \"syndromic.\" The underlying genetic defect may prone patients to develop hematopoietic diseases such as leukemia. Over the last decade, accumulating knowledge in genetics has led to the detection of many \"new\" platelet disorders. However, still many patients with a well-described platelet dysfunction remain undetected until severe bleeding occurs.</p>","PeriodicalId":55074,"journal":{"name":"Hamostaseologie","volume":null,"pages":null},"PeriodicalIF":2.7000,"publicationDate":"2023-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"Progress in Hemostasis (Part 1): Improved Management of Inherited Platelet Disorders: Reality or Illusion?\",\"authors\":\"Werner Streif\",\"doi\":\"10.1055/a-2031-7790\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><p>Platelets are key drivers of hemostasis. Low platelet counts, dysfunction in platelet adhesion, and aggregation lead to increased bleeding tendency. Inherited platelet disorders (IPDs) form a highly heterogeneous group of rare diseases with variable bleeding tendency. IPDs may be associated with other signs and symptoms often referred to as \\\"syndromic.\\\" The underlying genetic defect may prone patients to develop hematopoietic diseases such as leukemia. Over the last decade, accumulating knowledge in genetics has led to the detection of many \\\"new\\\" platelet disorders. However, still many patients with a well-described platelet dysfunction remain undetected until severe bleeding occurs.</p>\",\"PeriodicalId\":55074,\"journal\":{\"name\":\"Hamostaseologie\",\"volume\":null,\"pages\":null},\"PeriodicalIF\":2.7000,\"publicationDate\":\"2023-08-01\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Hamostaseologie\",\"FirstCategoryId\":\"3\",\"ListUrlMain\":\"https://doi.org/10.1055/a-2031-7790\",\"RegionNum\":4,\"RegionCategory\":\"医学\",\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"Q2\",\"JCRName\":\"HEMATOLOGY\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Hamostaseologie","FirstCategoryId":"3","ListUrlMain":"https://doi.org/10.1055/a-2031-7790","RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q2","JCRName":"HEMATOLOGY","Score":null,"Total":0}
引用次数: 0

摘要

血小板是止血的关键驱动因素。血小板计数低,血小板粘附功能障碍和聚集导致出血倾向增加。遗传性血小板疾病(IPDs)是一种异质性很强的罕见疾病,具有不同的出血倾向。ipd可能与其他通常被称为“综合征”的体征和症状有关。潜在的遗传缺陷可能使患者易患造血疾病,如白血病。在过去的十年中,遗传学知识的积累导致了许多“新的”血小板疾病的检测。然而,仍有许多有明确描述的血小板功能障碍的患者直到发生严重出血才被发现。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Progress in Hemostasis (Part 1): Improved Management of Inherited Platelet Disorders: Reality or Illusion?

Platelets are key drivers of hemostasis. Low platelet counts, dysfunction in platelet adhesion, and aggregation lead to increased bleeding tendency. Inherited platelet disorders (IPDs) form a highly heterogeneous group of rare diseases with variable bleeding tendency. IPDs may be associated with other signs and symptoms often referred to as "syndromic." The underlying genetic defect may prone patients to develop hematopoietic diseases such as leukemia. Over the last decade, accumulating knowledge in genetics has led to the detection of many "new" platelet disorders. However, still many patients with a well-described platelet dysfunction remain undetected until severe bleeding occurs.

求助全文
通过发布文献求助,成功后即可免费获取论文全文。 去求助
来源期刊
Hamostaseologie
Hamostaseologie HEMATOLOGY-
CiteScore
5.50
自引率
6.20%
发文量
62
审稿时长
6-12 weeks
期刊介绍: Hämostaseologie is an interdisciplinary specialist journal on the complex topics of haemorrhages and thromboembolism and is aimed not only at haematologists, but also at a wide range of specialists from clinic and practice. The readership consequently includes both specialists for internal medicine as well as for surgical diseases.
×
引用
GB/T 7714-2015
复制
MLA
复制
APA
复制
导出至
BibTeX EndNote RefMan NoteFirst NoteExpress
×
提示
您的信息不完整,为了账户安全,请先补充。
现在去补充
×
提示
您因"违规操作"
具体请查看互助需知
我知道了
×
提示
确定
请完成安全验证×
copy
已复制链接
快去分享给好友吧!
我知道了
右上角分享
点击右上角分享
0
联系我们:info@booksci.cn Book学术提供免费学术资源搜索服务,方便国内外学者检索中英文文献。致力于提供最便捷和优质的服务体验。 Copyright © 2023 布克学术 All rights reserved.
京ICP备2023020795号-1
ghs 京公网安备 11010802042870号
Book学术文献互助
Book学术文献互助群
群 号:481959085
Book学术官方微信