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Revel-Vilk S, Naamad M, Frydman D, et al. Platelet Activation and Reactivity in a Large Cohort of Patients with Gaucher Disease. Thromb Haemost. 2021;doi: 10.1055/a-1642-4206.
Revel-Vilk, S., Naamad, M., Frydman, D., Freund, M. R., Dinur, T., Istaiti, M., Becker-Cohen, M., Falk, R., Broide, E., Michelson, A. D., Frelinger, A. L., & Zimran, A. (2021). Platelet Activation and Reactivity in a Large Cohort of Patients with Gaucher Disease. Thrombosis and haemostasis, . https://doi.org/10.1055/a-1642-4206
Revel-Vilk, Shoshana, et al. "Platelet Activation and Reactivity in a Large Cohort of Patients with Gaucher Disease." Thrombosis and haemostasis vol. (2021). doi: https://doi.org/10.1055/a-1642-4206
Revel-Vilk S, Naamad M, Frydman D, Freund MR, Dinur T, Istaiti M, Becker-Cohen M, Falk R, Broide E, Michelson AD, Frelinger AL, Zimran A. Platelet Activation and Reactivity in a Large Cohort of Patients with Gaucher Disease. Thromb Haemost. 2021 Sep 10; doi: 10.1055/a-1642-4206. Epub 2021 Sep 10. PMID: 34507369.
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Thromb Haemost. 2021 Sep 10; doi: 10.1055/a-1642-4206. Epub 2021 Sep 10.

Platelet Activation and Reactivity in a Large Cohort of Patients with Gaucher Disease.

Thrombosis and haemostasis

Shoshana Revel-Vilk, Mira Naamad, Dafna Frydman, Michael R Freund, Tama Dinur, Majdolen Istaiti, Michal Becker-Cohen, Roni Falk, Eti Broide, Alan D Michelson, Andrew L Frelinger, Ari Zimran

Affiliations

  1. Gaucher Unit, Shaare Zedek Medical Center, Jerusalem, Israel.
  2. Pediatric Hematology/Oncology Unit, Shaare Zedek Medical Center, Jerusalem, Israel.
  3. Faculty of Medicine, Hebrew University of Jerusalem, Jerusalem, Israel.
  4. Flow Cytometry Unit, Shaare Zedek Medical Center, Jerusalem, Israel.
  5. Center for Platelet Research Studies, Dana-Farber/Boston Children's Cancer and Blood Disorders Center, Harvard Medical School, Boston, United States.

PMID: 34507369 DOI: 10.1055/a-1642-4206

Abstract

OBJECTIVES:  Patients 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.

METHODS:  Unstimulated and stimulated platelet function was measured by whole blood flow cytometry of platelet surface-activated αIIbβ3 integrin (detected with monoclonal antibody PAC1), P-selectin (CD62P), and lysosomal-associated membrane protein (LAMP3/CD63) in 149 GD patients.

RESULTS:  GD patients had a higher level of unstimulated CD63 expression than healthy subjects, which was mildly correlated with glucosylsphingosine (lyso-Gb1) levels (

CONCLUSION:  Flow cytometry enables the study of platelet function in thrombocytopenic GD patients. A platelet degranulation defect, but not αIIbβ3 integrin activation defect, is associated with clinical bleeding. In vivo increased CD63 expression may be related to GD-related inflammation.

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Conflict of interest statement

S.R.-V.: honoraria and research funding from Takeda, Pfizer, Sanofi/Genzyme. M.N., D.F., T.D., M.I., M.B.-C.: no conflict of interest to declare. A.D.M.: honoraria: AstraZeneca, Stasys. Research fundi

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