eutrOphil acTivity and dIversity (phenotype) in Sickle Cell disEase and their changes after treatment; The NOTICE study

Recruiting

Conditions: Hereditary blooddisorder
Sicklecell anaemia
10018902

Registration Number: NL-OMON52543

Lead Sponsor: Academisch Medisch Centrum

Brief Summary: Not available

Detailed Description: Not available

Recruitment & Eligibility

Status: Recruiting

Sex: Not specified

Target Recruitment: 90

Inclusion Criteria

1.Sickle cell disease patients (age >= 6 years) with high performance liquid
chromatography (HPLC) confirmed diagnosis of HbSS, HbSβ0-thalassemia, HbSC or
HbSβ+- thalassemia genotype
2. Willing and able to provide written informed consent
3. For the steady state/transfusion/hydroxyurea subgroup: visiting outpatient
clinic
4. For the subgroup of patients during painful crisis: inclusion should be
performed within 36 hours of admission to the hospital

Exclusion Criteria

1. Unable to sign informed consent
2. VOC within 4 weeks of the outpatient clinic visit (steady state subgroup)
3. Pregnancy
4. Active cancer
5. Chronic HIV infection
6. Use of immunosuppressive drugs

Study & Design

Study Type: Observational invasive

Study Design: Not specified

Primary Outcome Measures

Name	Time	Method
<p>Primary endpoint of this studie is the difference in activity and phenotypes of<br /><br>neutrophils in patients with sickle cell disease compared to those of healthy<br /><br>controls.<br /><br>We aim to address the following questions:<br /><br>- Is neutrophil activity (as measured by flow cytometry and in vitro release of<br /><br>NETs) altered in patients with SCD and does this differ in various<br /><br>circumstances, such as VOC/steady state?<br /><br>- Which subtypes (anti-inflammatory/pro-angiogenic (N2) vs. pro-inflammatory<br /><br>(N1)) of neutrophils are present in steady state SCD? Is there a relation with<br /><br>hemolytic vs. vaso-occlusive phenotype of SCD?<br /><br>- How does the neutrophil phenotype alter during vaso-occlusive crisis with<br /><br>increased inflammation and hypoxia as a result of this vaso-occlusion?</p><br>

Secondary Outcome Measures