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Elevated circulating endothelial membrane microparticles in paroxysmal nocturnal haemoglobinuria
Jan Simak1, Karel Holada, Antonio M Risitano
1Laboratory of Cellular Hematology, Division of Hematology, Center for Biologics Evaluation and Research, Food and Drug Administration, Bethesda, MD, USA. jan.simak@fda.hhs.gov
British Journal of Haematology
|June 8, 2004
Summary
Endothelial cell membrane microparticles (ECMP) are elevated in paroxysmal nocturnal haemoglobinuria (PNH) and sickle cell disease (SCD) patients, indicating vascular endothelium inflammation and injury. This finding may offer insights into disease status.
Area of Science:
- Hematology
- Vascular Biology
- Immunology
Background:
- Endothelial cell membrane microparticles (ECMP) are biomarkers of endothelial activation and injury.
- Paroxysmal nocturnal haemoglobinuria (PNH), aplastic anaemia (AA), and sickle cell disease (SCD) are hematologic disorders with potential vascular complications.
Purpose of the Study:
- To investigate the levels of circulating ECMP in patients with PNH, AA, and SCD compared to healthy donors (HD).
- To assess the potential of ECMP as indicators of endothelial cell status in these hematologic conditions.
Main Methods:
- Peripheral blood samples were collected from patients with PNH (n=9), AA (n=10), SCD (n=8), and HD (n=11).
- Three-color flow cytometry was used to analyze ECMP, specifically CD105+ and CD144+ microparticles, with annexin V-binding (AVB) assay for phosphatidylserine exposure.
- Antibodies to endothelial antigens CD105, CD144, and inflammatory marker CD54 were employed.
Main Results:
- Elevated levels of CD105+AVB+ ECMP were observed in SCD and PNH patients compared to AA and HD groups (P < 0.05).
- ECMP expressing CD54 (inflammatory marker) and CD144 (endothelial marker) were significantly increased in SCD and PNH patients.
- AA patients showed no significant elevation in these ECMP populations compared to healthy donors.
Conclusions:
- Circulating ECMP levels, particularly CD54+ and CD144+ populations, are elevated in SCD and PNH, reflecting endothelial inflammation and injury.
- Analysis of ECMP shows promise for monitoring the vascular endothelium status in PNH and SCD patients.