伊朗输血组织专门凝血实验室转诊患者血小板分泌失调的频率、临床和实验室结果。

IF 1.2 4区 医学 Q4 HEMATOLOGY
Blood Coagulation & Fibrinolysis Pub Date : 2024-04-01 Epub Date: 2024-03-11 DOI:10.1097/MBC.0000000000001289
Massoumeh Shahbazi, Minoo Ahmadinejad, Vahid Pirhajati Mahabadi, Amir Teimourpour, Khadijeh Golzadeh
{"title":"伊朗输血组织专门凝血实验室转诊患者血小板分泌失调的频率、临床和实验室结果。","authors":"Massoumeh Shahbazi, Minoo Ahmadinejad, Vahid Pirhajati Mahabadi, Amir Teimourpour, Khadijeh Golzadeh","doi":"10.1097/MBC.0000000000001289","DOIUrl":null,"url":null,"abstract":"<p><strong>Objectives: </strong>Platelet secretion disorders (PSDs) are a subgroup of platelet function disorders (PFDs) caused by defects in the content or release of platelet granules. These patients have a variable degree of mucocutaneous bleeding tendency. The diagnostic facilities of PSDs are imitated in Iran, even in specialized coagulation laboratories. The present study aims to estimate the frequency of PSDs among patients referred to the Iranian Blood Transfusion Organization (IBTO).</p><p><strong>Methods: </strong>The research population includes all patients referred to the specialized coagulation laboratory of IBTO and requested platelet function and von Willebrand testing by their physicians. They were recruited between May 2022 and October 2022 if they were not diagnosed as having procoagulant defects, von Willebrand disease (VWD), Glanzmann thrombasthenia (GT), Bernard-Soulier syndrome (BSS), and platelet count <100 × 10 9 (except in the syndromic forms). Patients with a defect in response to at least two agonists in Light transmission aggregometry (LTA), one agonist in the ATP-secretion study, and/or impairment in the expression of CD62P are considered PSDs.</p><p><strong>Results: </strong>Among 121 cases referred to our center over 6 months, 40 patients fulfilled the inclusion and exclusion criteria. Ten patients were diagnosed with PSDs. Six were classified as δ-platelet secretion disorders (δ-PSD), two α-platelet secretion disorders (α-PSD), and two αδ-platelet secretion disorders (αδ-PSD).</p><p><strong>Conclusions: </strong>The prevalence of PSDs in our population study was 25% (10/40), which seems highly prevalent. Therefore, expanding laboratory approaches to platelet function defects is necessary as a routine in our country.</p>","PeriodicalId":8992,"journal":{"name":"Blood Coagulation & Fibrinolysis","volume":null,"pages":null},"PeriodicalIF":1.2000,"publicationDate":"2024-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"Frequency, clinical, and laboratory findings of platelet secretion disorders in patients referred to the specialized coagulation laboratory of the Iranian Blood Transfusion Organization.\",\"authors\":\"Massoumeh Shahbazi, Minoo Ahmadinejad, Vahid Pirhajati Mahabadi, Amir Teimourpour, Khadijeh Golzadeh\",\"doi\":\"10.1097/MBC.0000000000001289\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><strong>Objectives: </strong>Platelet secretion disorders (PSDs) are a subgroup of platelet function disorders (PFDs) caused by defects in the content or release of platelet granules. These patients have a variable degree of mucocutaneous bleeding tendency. The diagnostic facilities of PSDs are imitated in Iran, even in specialized coagulation laboratories. The present study aims to estimate the frequency of PSDs among patients referred to the Iranian Blood Transfusion Organization (IBTO).</p><p><strong>Methods: </strong>The research population includes all patients referred to the specialized coagulation laboratory of IBTO and requested platelet function and von Willebrand testing by their physicians. They were recruited between May 2022 and October 2022 if they were not diagnosed as having procoagulant defects, von Willebrand disease (VWD), Glanzmann thrombasthenia (GT), Bernard-Soulier syndrome (BSS), and platelet count <100 × 10 9 (except in the syndromic forms). Patients with a defect in response to at least two agonists in Light transmission aggregometry (LTA), one agonist in the ATP-secretion study, and/or impairment in the expression of CD62P are considered PSDs.</p><p><strong>Results: </strong>Among 121 cases referred to our center over 6 months, 40 patients fulfilled the inclusion and exclusion criteria. Ten patients were diagnosed with PSDs. Six were classified as δ-platelet secretion disorders (δ-PSD), two α-platelet secretion disorders (α-PSD), and two αδ-platelet secretion disorders (αδ-PSD).</p><p><strong>Conclusions: </strong>The prevalence of PSDs in our population study was 25% (10/40), which seems highly prevalent. Therefore, expanding laboratory approaches to platelet function defects is necessary as a routine in our country.</p>\",\"PeriodicalId\":8992,\"journal\":{\"name\":\"Blood Coagulation & Fibrinolysis\",\"volume\":null,\"pages\":null},\"PeriodicalIF\":1.2000,\"publicationDate\":\"2024-04-01\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Blood Coagulation & Fibrinolysis\",\"FirstCategoryId\":\"3\",\"ListUrlMain\":\"https://doi.org/10.1097/MBC.0000000000001289\",\"RegionNum\":4,\"RegionCategory\":\"医学\",\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"2024/3/11 0:00:00\",\"PubModel\":\"Epub\",\"JCR\":\"Q4\",\"JCRName\":\"HEMATOLOGY\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Blood Coagulation & Fibrinolysis","FirstCategoryId":"3","ListUrlMain":"https://doi.org/10.1097/MBC.0000000000001289","RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"2024/3/11 0:00:00","PubModel":"Epub","JCR":"Q4","JCRName":"HEMATOLOGY","Score":null,"Total":0}
引用次数: 0

摘要

目的:血小板分泌障碍(PSDs)是血小板功能障碍(PFDs)的一个亚组,由血小板颗粒的含量或释放缺陷引起。这些患者有不同程度的粘膜出血倾向。在伊朗,即使在专门的凝血实验室,PSD 的诊断设备也是仿制的。本研究旨在估算转诊至伊朗输血组织(IBTO)的患者中 PSD 的发生频率:研究对象包括所有转诊至伊朗输血组织(IBTO)专业凝血实验室并由医生要求进行血小板功能和 von Willebrand 检测的患者。方法:研究对象包括所有转诊至 IBTO 专门凝血实验室并由医生要求进行血小板功能和冯-维勒布兰德检测的患者,时间为 2022 年 5 月至 2022 年 10 月,条件是他们未被诊断出患有促凝血缺陷、冯-维勒布兰德病(VWD)、格兰兹曼血栓形成症(GT)、伯纳德-苏利尔综合征(BSS)和血小板计数结果:在 6 个月内转诊至本中心的 121 例患者中,有 40 例符合纳入和排除标准。10名患者被诊断为 PSD。6例被归类为δ-血小板分泌障碍(δ-PSD),2例为α-血小板分泌障碍(α-PSD),2例为αδ-血小板分泌障碍(αδ-PSD):结论:在我们的人群研究中,PSD 的发病率为 25%(10/40),似乎非常普遍。因此,在我国有必要将扩大血小板功能缺陷的实验室方法作为一项常规工作。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Frequency, clinical, and laboratory findings of platelet secretion disorders in patients referred to the specialized coagulation laboratory of the Iranian Blood Transfusion Organization.

Objectives: Platelet secretion disorders (PSDs) are a subgroup of platelet function disorders (PFDs) caused by defects in the content or release of platelet granules. These patients have a variable degree of mucocutaneous bleeding tendency. The diagnostic facilities of PSDs are imitated in Iran, even in specialized coagulation laboratories. The present study aims to estimate the frequency of PSDs among patients referred to the Iranian Blood Transfusion Organization (IBTO).

Methods: The research population includes all patients referred to the specialized coagulation laboratory of IBTO and requested platelet function and von Willebrand testing by their physicians. They were recruited between May 2022 and October 2022 if they were not diagnosed as having procoagulant defects, von Willebrand disease (VWD), Glanzmann thrombasthenia (GT), Bernard-Soulier syndrome (BSS), and platelet count <100 × 10 9 (except in the syndromic forms). Patients with a defect in response to at least two agonists in Light transmission aggregometry (LTA), one agonist in the ATP-secretion study, and/or impairment in the expression of CD62P are considered PSDs.

Results: Among 121 cases referred to our center over 6 months, 40 patients fulfilled the inclusion and exclusion criteria. Ten patients were diagnosed with PSDs. Six were classified as δ-platelet secretion disorders (δ-PSD), two α-platelet secretion disorders (α-PSD), and two αδ-platelet secretion disorders (αδ-PSD).

Conclusions: The prevalence of PSDs in our population study was 25% (10/40), which seems highly prevalent. Therefore, expanding laboratory approaches to platelet function defects is necessary as a routine in our country.

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
CiteScore
1.90
自引率
0.00%
发文量
111
审稿时长
4-8 weeks
期刊介绍: Blood Coagulation & Fibrinolysis is an international fully refereed journal that features review and original research articles on all clinical, laboratory and experimental aspects of haemostasis and thrombosis. The journal is devoted to publishing significant developments worldwide in the field of blood coagulation, fibrinolysis, thrombosis, platelets and the kininogen-kinin system, as well as dealing with those aspects of blood rheology relevant to haemostasis and the effects of drugs on haemostatic components
×
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