Hereditary Spherocytosis and Vascular Function

Hereditary Spherocytosis Clinical Trial

— VASCUSPHERO  

Official title:

Relationships Between Hemolysis, Erythrosis, Circulating Microparticles and Vascular Function in Patients With Hereditary Spherocytosis

Clinical Trial Details — Status: Completed

Administrative data

• NCT number: NCT04451785
• Other study ID #: 69HCL19_0949
• Secondary ID: 2019-A03203-54
• Status: Completed
• Phase: N/A
• Start date: August 26, 2020
• Est. completion date: August 26, 2022

Study information

• Verified date: April 2024
• Source: Hospices Civils de Lyon
• Contact: n/a
• Is FDA regulated: No
• Study type: Interventional

Clinical Trial Summary

Background : Patients with hereditary spherocytosis (HS) are characterized by increased red blood cell (RBC) fragility and a loss of RBC deformability. While the clinical variability of the disease may be heterogenous from one patient to another, some studies reported the occurrence of vascular complications, notably in patients who have been splenectomized.

Purpose : The aim of the study is to test the associations between the degree of vascular dysfunction and the extent of hemolysis, the amount of circulating microparticles, the level of erythrosis and the degree of RBC biophysical alterations.

Abstract : Recent studies reported the occurrence of vascular complications in patients with HS, notably in patients who have previously been splenectomized. However, the exact reasons of these complications are unknown and no study investigated the vascular function in HS patients.

Main objective Highlight the presence of altered vascular function in HS patients and test the relationships with the level of hemolysis and circulating microparticles.

Secondary objectives To evaluate the associations between clinical severity and 1) the level of vascular dysfunction and 2) several biomarkers (hemolysis, hematological parameters, circulating microparticles, erythrosis, RBC biophysical properties).

Recruitment information / eligibility

• Status: Completed
• Enrollment: 72
• Est. completion date: August 26, 2022
• Est. primary completion date: August 26, 2022
• Accepts healthy volunteers: Accepts Healthy Volunteers
• Gender: All
• Age group: 6 Years and older

Eligibility

Inclusion Criteria:

Healthy controls:

• age = 6 years old

• written, informed and signed consent by the healthy volunteer, or by both parents or legal guardian if the healthy volunteer is a minor

• Healthy volunteer affiliated to a social security scheme or assimilated

• Healthy volunteer not subject to any legal protection measure

Patients with hereditary spherocytosis:

• age = 6 years old

• Patient with hereditary or non-splenectomized spherocytosis

• Written, informed and signed consent by the patient, or by at least one of the two parents or legal guardian if the patient is a minor

• Patient affiliated to a social security scheme or assimilated

• Patient not subject to any legal protection measure

Exclusion Criteria:

Healthy controls:

• Pregnant or lactating woman

• Subjects with hereditary spherocytosis or other characterized condition by chronic hemolysis

• Subjects with known pathology affecting the vascular system

• Blood donation (less than a month old)

• Not affiliated to a social security scheme

• Patient participating in another interventional research protocol that may interfere with this protocol (according to the investigator's judgment).

Patients with hereditary spherocytosis:

• Patient who received a blood transfusion in the 3 months preceding

• Pregnant or lactating woman

• Any disease or condition other than hereditary spherocytosis, chronic or not, likely to induce chronic or acute intravascular hemolysis

• Patient participating in another interventional research protocol that may interfere with this protocol (according to the investigator's judgment).

Related Conditions & MeSH terms

• Hereditary Spherocytosis

Intervention

Biological:
• blood sample
6 tubes of 4 milliliters (ml) maximum (total: 24 ml) will be sampled for the measurements of the different biological markers. In case of the genetic mutation is already known, only 5 tubes will be collected (total: 20 ml).

Diagnostic Test:
• Pulse wave velocity
Non-invasive measurement of pulse wave velocity between the carotid and femoral arteries with piezo-electric sensors.

Locations

Country	Name	City	State
France	Hôpital Edouard Herriot	Lyon
France	Institut d'Hématologie et Oncologie PEdiatrique (iHOPe)	Lyon

Sponsors (1)

Lead Sponsor	Collaborator
Hospices Civils de Lyon

Country where clinical trial is conducted

France, 

Outcome

Type	Measure	Description	Time frame	Safety issue
Primary	measurement of pulse wave velocity (PWV)	Vascular function (arterial stiffness) will be investigated by the measurement of pulse wave velocity (PWV). Vascular dysfunction will be defined by a PWV value higher than 6 meter/second (m/s) and 10 m/s in children and adults with HS, respectively.	Day 1
Secondary	Hemogram	The hemogram is a complete blood count of the different cell types. All these measures are performed simultaneously on a standard hematology analyzer.	Day 1
Secondary	Markers of hemolysis	These markers are measured simultaneously on a standard biochemistry analyzer	Day 1
Secondary	Circulating microparticles	Circulating microparticles of various cell origin will be measured by flow cytometry	Day 1
Secondary	Markers of erythrosis	Markers of erythrosis (i.e., suicidal death of red blood cells) will be measured by flow cytometry	Day 1
Secondary	Blood viscosity	Blood viscosity (expressed in Pa.s) will be measured on a cone-plate viscosimeter, ektacytometry and light transmission, respectively.	Day 1
Secondary	Red blood cell deformability	Red blood cell deformability will be measured simultaneously on a Lorrca ektacytometry (Laser-assisted Rotational Red Cell Analyser).	Day 1
Secondary	Red blood cell aggregation	Red blood cell aggregation will be measured simultaneously on a Lorrca ektacytometry (Laser-assisted Rotational Red Cell Analyser).	Day 1