Related Experiment Video
Updated: Jun 24, 2026
Endothelialized Microfluidics for Studying Microvascular Interactions in Hematologic Diseases
Published on: June 22, 2012
Circulating endothelial cells: a potential parameter of organ damage in sickle cell anemia?
Michiel H Strijbos1, Precious P Landburg, Erfan Nur
1Department of Medical Oncology, Erasmus Medical Center, Rotterdam, The Netherlands.
Insights
Elevated circulating endothelial cells (CECs) indicate vascular injury in sickle cell disease (SCD). Higher CEC counts in asymptomatic SCD patients correlate with organ damage, suggesting CECs as a key biomarker for monitoring disease progression.
Area of Science:
- Hematology
- Vascular Biology
- Biomarker Discovery
Background:
- Sickle cell disease (SCD) requires objective tools to monitor organ damage.
- Circulating endothelial cells (CECs) are indicators of vascular injury.
Purpose of the Study:
- To detect elevated CECs in asymptomatic SCD patients using the CellSearch system.
- To investigate the relationship between CEC counts and clinical/biomarker indicators of SCD severity.
Main Methods:
- Analyzed CEC counts in 15 asymptomatic HbSS patients and 15 HbAA controls.
- Assessed laboratory parameters: Hb, leukocyte counts, HbF%, sVCAM-1, VWF:Ag, and ADMA.
- Correlated CEC counts with clinical data, including pulmonary hypertension and organ involvement.
Main Results:
- CEC counts were significantly higher in SCD patients (12 cells/mL) than controls (4 cells/mL) (P=0.007).
- Elevated CEC counts were associated with pulmonary hypertension (P=0.015) and increased organ damage (P=0.002).
- No significant correlations were found between CEC counts and other laboratory parameters.
Conclusions:
- CECs are elevated in asymptomatic SCD patients, indicating ongoing vascular injury.
- CEC counts serve as a potential biomarker for assessing vasculopathy and organ damage in SCD.
- The CellSearch system can effectively quantify CECs for SCD monitoring.
Abstract:
Objective laboratory tools are needed to monitor developing organ damage in sickle cell disease (SCD). Circulating endothelial cells (CECs) are indicative of vascular injury. We determined whether elevated CEC can be detected in asymptomatic SCD with the CellSearch system and whether the CEC count is related to clinical and blood-based biomarkers of disease severity. Fifteen consecutive clinically asymptomatic HbSS patients and 15 matched HbAA controls were analyzed for CEC counts, laboratory parameters of disease severity (Hb, leukocyte counts, HbF%), plasma levels of markers for endothelial activation (sVCAM-1, VWF:Ag) and of endogenous inhibitors of nitric oxide synthase (asymmetrical dimethylarginine [ADMA]). CEC counts were significantly higher in patients (12 cells/mL, IQR 8-29) as compared to controls (4 cells/mL, 3-10) (P=0.007). CEC counts were significantly higher in patients with pulmonary hypertension (PHT) (P=0.015), and increased with increasing number of affected organs (0-4 involved organs, P=0.002). No significant correlations between CEC and any other laboratory parameter were detected. In conclusion, CECs could prove to be an important new tool for assessing developing vasculopathy and organ damage in SCD.
