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Immunophenotypic Profile of Peripheral Blood Mononuclear Cells and T cell Function in Sickle Cell Disease Patients.

Recruiting
Conditions
hemoglobinopathy
Sickle cell disease
10018902
Registration Number
NL-OMON54195
Lead Sponsor
Academisch Medisch Centrum
Brief Summary

Not available

Detailed Description

Not available

Recruitment & Eligibility

Status
Recruiting
Sex
Not specified
Target Recruitment
60
Inclusion Criteria

Inclusion criteria SCD patients
To be eligible to participate in this study, a subject must meet all the
following criteria:
o Age 12 years or older
o High performance liquid chromatography (HPLC) confirmed diagnosis of
HbSS/HbSß0
o Willing and able to provide written informed consent
o For children 12-16 years: additional written informed consent of
parent/caretaker

Inclusion criteria healthy volunteers
To be eligible to participate in this study, a subject must meet all the
following criteria:
o Age 18 years or older
o Willing and able to provide informed consent
o No self-reported medical issues that could interfere with immunity
o Originating (or parents originating) from region where SCD occurs

Exclusion Criteria

Exclusion criteria SCD patients
A potential subject who meets any of the following criteria will be excluded
from participation in this study:
o History of allogeneic SCT
o Participation in Amsterdam UMC SCT biobank because of planned allogeneic SCT
o History of chronic infection or autoimmune inflammatory disease
o Pregnancy, self-reported
o Vaso-occlusive disease in the preceding 4 weeks, self-reported
o Red blood cell transfusion in the preceding 3 months, self-reported

Exclusion criteria for healthy volunteers
A potential subject who meets any of the following criteria will be excluded
from participation in this study:
o Carriership of sickle cell gene or other hemoglobinopathy, self-reported
o Chronic infection, self-reported
o Chronic auto-inflammatory disease, self-reported
o Use of chronic medication that affects the immune system, self-reported

Study & Design

Study Type
Observational invasive
Study Design
Not specified
Primary Outcome Measures
NameTimeMethod
<p>The characterization of PBMC subsets in SCD patients with the HbSS/HbSß0<br /><br>genotype as compared to matched healthy controls in order to elucidate<br /><br>potential perturbations in the adaptive immunity (PBMC subsets) in SCD.</p><br>
Secondary Outcome Measures
NameTimeMethod
<p>The identification of differences between the immunophenotypic profile of PBMC<br /><br>subsets of adolescent SCD patients compared to matched healthy controls.<br /><br>The identification of differences between the immunophenotypic profile of PBMC<br /><br>subsets of adult and adolescent HbSS/HbSß0 genotype SCD patients.<br /><br>The determination of general T-cell effector function in steady state SCD<br /><br>patients in order to establish a reference that can be used in further research<br /><br>evaluation T-cell function after allogeneic SCT for SCD.</p><br>
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