{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,23]],"date-time":"2026-09-23T20:59:35Z","timestamp":1790197175386,"version":"4.1.0"},"reference-count":41,"publisher":"Georg Thieme Verlag KG","issue":"06","funder":[{"name":"Pfizer Israel"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Thromb Haemost"],"published-print":{"date-parts":[[2022,6]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>\n          Objectives\u2003Patients with Gaucher disease (GD) are at increased risk of bleeding and have varying degrees of thrombocytopenia, making the analysis of platelet function difficult. This study aimed to provide a clinically relevant quantitative assessment of platelet function and determine its relationship with bleeding and GD-related data.<\/jats:p><jats:p>\n          Methods\u2003Unstimulated and stimulated platelet function was measured by whole blood flow cytometry of platelet surface-activated \u03b1IIb\u03b23 integrin (detected with monoclonal antibody PAC1), P-selectin (CD62P), and lysosomal-associated membrane protein (LAMP3\/CD63) in 149 GD patients.<\/jats:p><jats:p>\n          Results\u2003GD patients had a higher level of unstimulated CD63 expression than healthy subjects, which was mildly correlated with glucosylsphingosine (lyso-Gb1) levels (r\u2009=\u20090.17, p-value\u2009=\u20090.042). Splenectomized GD patients had a higher level of unstimulated \u03b1IIb\u03b23 integrin and P-selectin expression. Reduced platelet reactivity (\u22122 standard deviation of reference range) was found in 79 (53%, 95% confidence interval [CI]: 44\u201361%) patients, of whom 10 (6.7%, 95% CI: 3.3\u201312%) had more severe platelet dysfunction. In a multivariate model, only lyso-Gb1 levels were associated with the more severe platelet dysfunction. Fifty-four (49%) of 128 adult patients who completed the bleeding tendency questionnaire reported positive bleeding history. In a multivariate logistic model, older age (odds ratio [OR]: 1.05, 95% CI: 1.01\u20131.1) and low P-selectin reactivity (OR: 2.03, 95% CI: 1.25\u20133.35) were associated with more than one bleeding manifestation.<\/jats:p><jats:p>\n          Conclusion\u2003Flow cytometry enables the study of platelet function in thrombocytopenic GD patients. A platelet degranulation defect, but not \u03b1IIb\u03b23 integrin activation defect, is associated with clinical bleeding. In vivo increased CD63 expression may be related to GD-related inflammation.<\/jats:p>","DOI":"10.1055\/a-1642-4206","type":"journal-article","created":{"date-parts":[[2021,9,10]],"date-time":"2021-09-10T23:13:05Z","timestamp":1631315585000},"page":"951-960","source":"Crossref","is-referenced-by-count":10,"title":["Platelet Activation and Reactivity in a Large Cohort of Patients with Gaucher Disease"],"prefix":"10.1055","volume":"122","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-9151-0337","authenticated-orcid":false,"given":"Shoshana","family":"Revel-Vilk","sequence":"additional","affiliation":[{"name":"Gaucher Unit, Shaare Zedek Medical Center, Jerusalem, Israel"},{"name":"Pediatric Hematology\/Oncology Unit, Shaare Zedek Medical Center, Jerusalem, Israel"},{"name":"Faculty of Medicine, Hebrew University of Jerusalem, Jerusalem, Israel"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mira","family":"Naamad","sequence":"additional","affiliation":[{"name":"Flow Cytometry Unit, Shaare Zedek Medical Center, Jerusalem, Israel"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dafna","family":"Frydman","sequence":"additional","affiliation":[{"name":"Gaucher Unit, Shaare Zedek Medical Center, Jerusalem, Israel"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michael R.","family":"Freund","sequence":"additional","affiliation":[{"name":"Gaucher Unit, Shaare Zedek Medical Center, Jerusalem, Israel"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tama","family":"Dinur","sequence":"additional","affiliation":[{"name":"Gaucher Unit, Shaare Zedek Medical Center, Jerusalem, Israel"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Majdolen","family":"Istaiti","sequence":"additional","affiliation":[{"name":"Gaucher Unit, Shaare Zedek Medical Center, Jerusalem, Israel"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michal","family":"Becker-Cohen","sequence":"additional","affiliation":[{"name":"Gaucher Unit, Shaare Zedek Medical Center, Jerusalem, Israel"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Roni","family":"Falk","sequence":"additional","affiliation":[{"name":"Flow Cytometry Unit, Shaare Zedek Medical Center, Jerusalem, Israel"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Eti","family":"Broide","sequence":"additional","affiliation":[{"name":"Flow Cytometry Unit, Shaare Zedek Medical Center, Jerusalem, Israel"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alan D.","family":"Michelson","sequence":"additional","affiliation":[{"name":"Center for Platelet Research Studies, Dana-Farber\/Boston Children's Cancer and Blood Disorders Center, Harvard Medical School, Boston, United States"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"suffix":"III","given":"Andrew L.","family":"Frelinger","sequence":"additional","affiliation":[{"name":"Center for Platelet Research Studies, Dana-Farber\/Boston Children's Cancer and Blood Disorders Center, Harvard Medical School, Boston, United States"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ari","family":"Zimran","sequence":"additional","affiliation":[{"name":"Gaucher Unit, Shaare Zedek Medical Center, Jerusalem, Israel"},{"name":"Faculty of Medicine, Hebrew University of Jerusalem, Jerusalem, Israel"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"194","published-online":{"date-parts":[[2021,11,5]]},"reference":[{"key":"ref1","first-page":"1189","volume-title":"Gaucher disease and related lysosomal storage diseases","author":"S Revel-Vilk","year":"2021","edition":"10th ed."},{"issue":"02","key":"ref2","doi-asserted-by":"crossref","first-page":"143","DOI":"10.3109\/09537104.2011.597526","article-title":"Haemostatic abnormalities in treatment-na\u00efve patients with Type 1 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