دورية أكاديمية
أعرض في EDS

Levels of procoagulant microparticles expressing phosphatidylserine contribute to bleeding phenotype in patients with inherited thrombocytopenia.

التفاصيل البيبلوغرافية
العنوان: Levels of procoagulant microparticles expressing phosphatidylserine contribute to bleeding phenotype in patients with inherited thrombocytopenia.
المؤلفون: Brøns N; Department of Hematology.; Department of Clinical Immunology., Leinøe E; Department of Hematology., Salado-Jimena JA; Department of Clinical Immunology., Rossing M; Center for Genomic Medicine, Copenhagen University Hospital, Rigshospitalet, København, Denmark., Ostrowski SR; Department of Clinical Immunology.
المصدر: Blood coagulation & fibrinolysis : an international journal in haemostasis and thrombosis [Blood Coagul Fibrinolysis] 2021 Oct 01; Vol. 32 (7), pp. 480-490.
نوع المنشور: Journal Article
اللغة: English
بيانات الدورية: Publisher: Lippincott Williams And Wilkins Country of Publication: England NLM ID: 9102551 Publication Model: Print Cited Medium: Internet ISSN: 1473-5733 (Electronic) Linking ISSN: 09575235 NLM ISO Abbreviation: Blood Coagul Fibrinolysis Subsets: MEDLINE
أسماء مطبوعة: Publication: London : Lippincott Williams And Wilkins
Original Publication: Oxford, UK : Rapid Communications of Oxford Ltd., c1990-
مواضيع طبية MeSH: Cell-Derived Microparticles/*metabolism , Hemorrhage/*metabolism , Phosphatidylserines/*metabolism , Thrombocytopenia/*metabolism, Adult ; Aged ; Blood Coagulation ; Blood Platelets/cytology ; Blood Platelets/metabolism ; Female ; Hemorrhage/blood ; Hemorrhage/etiology ; Humans ; Male ; Middle Aged ; Phosphatidylserines/blood ; Platelet Activation ; Thrombocytopenia/blood ; Thrombocytopenia/complications ; Young Adult
مستخلص: Inherited thrombocytopenia is a heterogeneous group of hereditary disorders with varying bleeding tendencies, not simply related to platelet count. Platelets transform into different subpopulations upon stimulation, including procoagulant platelets and platelet microparticles (PMPs), which are considered critical for haemostasis. We aimed to investigate whether abnormalities in PMP and procoagulant platelet function were associated with the bleeding phenotype of inherited thrombocytopenia patients. We enrolled 53 inherited thrombocytopenia patients. High-throughput sequencing of 36 inherited thrombocytopenia related genes was performed in all patients and enabled a molecular diagnosis in 57%. Bleeding phenotype was evaluated using the ISTH bleeding assessment tool, dividing patients into bleeding (n = 27) vs. nonbleeding (n = 26). Unstimulated and ADP, TRAP or collagen-stimulated PMP and procoagulant platelet functions were analysed by flow cytometry using antibodies against granulophysin (CD63), P-selectin (CD62P), activated GPIIb/IIIa (PAC-1) and a marker for phosphatidylserine expression (lactadherin). Procoagulant platelets were measured in response to collagen stimulation. An in-house healthy reference level was available. Overall, higher levels of activated platelets, PMPs and procoagulant platelets were found in nonbleeding patients compared with the reference level. Nonbleeding patients had higher proportions of phosphatidylserine and PMPs compared with bleeding patients and the reference level, in response to different stimulations. Interestingly, this finding of high proportions of phosphatidylserine and PMPs was limited to PMPs, and not present in procoagulant platelets or platelets. Our findings indicate that nonbleeding inherited thrombocytopenia patients have compensatory mechanisms for improved platelet subpopulation activation and function, and that generation of phosphatidylserine expressing PMPs could be a factor determining bleeding phenotype in inherited thrombocytopenia.
(Copyright © 2021 Wolters Kluwer Health, Inc. All rights reserved.)
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المشرفين على المادة: 0 (Phosphatidylserines)
تواريخ الأحداث: Date Created: 20210903 Date Completed: 20211126 Latest Revision: 20230924
رمز التحديث: 20231215
DOI: 10.1097/MBC.0000000000001080
PMID: 34475331
قاعدة البيانات: MEDLINE
الوصف
تدمد:1473-5733
DOI:10.1097/MBC.0000000000001080