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Monocytic Expression of Heme Oxidase-1 (HO-1) in Sickle Cell Patients and Correlation With the Humoral Immune Response to Vaccine and With Allo-immunization.

Not Applicable
Completed
Conditions
Sickle Cell Disease
Interventions
Biological: Inactivated influenza A (H1N1) virus vaccine
Diagnostic Test: Blood sampling
Registration Number
NCT03111589
Lead Sponsor
Francis Corazza
Brief Summary

Sickle cell disease (SCD) is an autosomal recessive disorder resulting from a substitution in the β chain of hemoglobin (Hb) which causes hemoglobin S to polymerize when deoxygenated. SCD patients present immune abnormalities that have always been attributed to functional asplenia. It it is now being recognized that patients with SCD have a pro-inflammatory condition with altered immune system activation contributing to the pathology of SCD. Increased levels of neutrophils, monocytes or cytokines have been reported in SCD patients.

SCD is associated with many acute and chronic complications requiring immediate support. Actual strongly recommended therapies include chronic blood transfusions (CT) and hydroxyurea (HU). In addition, episodic transfusions are recommended and commonly used to manage many acute SCD complications.There is strong evidence to support the use of HU in adults with 3 or more severe vaso-occlusive crises during any 12-month period, with SCD pain or chronic anemia, or with severe or recurrent episodes of acute chest syndrome. HU use is now also common in children with SCD. Some patients receive chronic monthly RBC transfusion with the objective to reduce the proportion of HbS to \< 30 %. Long-term RBC transfusions prevent and treat complications of SCD decreasing the risk of stroke and the incidence of acute chest syndrome (ACS).

Therapeutic complications, such as alloimmunization against RBC in 20-50% of patients or hematopoietic stem cell transplantation (HSCT) graft rejection, constitute an immune-based clinical issue in SCD. Poorly understood RBC alloimmunization is responsible for serious hemolytic transfusion reaction associated with severe mortality and morbidity underlying the need for a better understanding of the immunology of SCD to improve SCD transfusion support/outcome. Little evidence exists about HU effects on immune functions in SCD. HU treatment doesn't appear to have deleterious effects on immune function and appears to decrease the abnormally elevated number of total WBC and lymphocytes, while CT does not.

Patients with SCD are at higher risk of infections and prophylactic vaccination is strongly recommended. Recent data suggest that vaccinal response to pneumococcal antigens in SCD patients is identical to healthy control while controversy concern the stability of the immune protection after vaccination of SCD patient. Antibody levels declined over the year and the need for more frequent vaccination in SCD patient should be investigated. Currently, there is no evidence whether HU may interfere with pneumococcal immune response. Purohit showed that immune response to inactivated influenza A (H1N1) virus vaccine was altered in patient with SCD receiving CT but little is known on immune response to vaccination in patients with SCD receiving HU.

Recent data suggest that not only inflammatory status but also humoral immune response to antigens in SCD patients may differ according to treatment. Yazdanbakhsh reported an imbalance between regulatory T cell (Treg) and effector T cell (Teff) in alloimmunized SCD patients with as consequence an increase in antibody production. In a model proposed by the authors, the balance between Treg and Teff is dictated by the monocyte control of cytokines expression. Altered activity of monocyte heme oxidase-1 (HO-1) would be responsible of a decrease in IL-12 and an increase in IL-10 cytokines secretion impacting the Treg/Teff cells ratio and promoting antibody production by B cells.

The objectives of the project are to assess whether different humoral immune responses to vaccines or to erythrocyte alloantigens are related to the type of treatment administered to patients with SCD. We also aim to study if these differences might be related to different expressions of HO-1 by monocytes.

Detailed Description

Not available

Recruitment & Eligibility

Status
COMPLETED
Sex
All
Target Recruitment
102
Inclusion Criteria
  • Pediatric and adult patients with sickle cell disease from the HUDERF and CHU-Brugmann Hospital
Exclusion Criteria
  • None

Study & Design

Study Type
INTERVENTIONAL
Study Design
PARALLEL
Arm && Interventions
GroupInterventionDescription
SCD patients under regular chronic exchange transfusionBlood samplingSickle cell disease patients (SCD) under regular chronic exchange transfusions. Pediatric and adult patients from the HUDERF and CHU-Brugmann Hospitals.
SCD patients under HU treatment+sporadic transfusionBlood samplingSickle cell disease patients (SCD) under hydroxyurea (HU) and receiving sporadic transfusions.Pediatric and adult patients from the HUDERF and CHU-Brugmann Hospitals.
SCD patients under regular chronic exchange transfusionInactivated influenza A (H1N1) virus vaccineSickle cell disease patients (SCD) under regular chronic exchange transfusions. Pediatric and adult patients from the HUDERF and CHU-Brugmann Hospitals.
SCD patients under HU treatment aloneInactivated influenza A (H1N1) virus vaccineSickle cell disease patients (SCD) under hydroxyurea (HU) alone. Pediatric and adult patients from the HUDERF and CHU-Brugmann Hospitals.
Control groupInactivated influenza A (H1N1) virus vaccinePediatric and adult patients from the HUDERF and CHU-Brugmann Hospitals.
SCD patients under HU treatment aloneBlood samplingSickle cell disease patients (SCD) under hydroxyurea (HU) alone. Pediatric and adult patients from the HUDERF and CHU-Brugmann Hospitals.
Control groupBlood samplingPediatric and adult patients from the HUDERF and CHU-Brugmann Hospitals.
SCD patients under HU treatment+sporadic transfusionInactivated influenza A (H1N1) virus vaccineSickle cell disease patients (SCD) under hydroxyurea (HU) and receiving sporadic transfusions.Pediatric and adult patients from the HUDERF and CHU-Brugmann Hospitals.
Primary Outcome Measures
NameTimeMethod
Cytokines levels measurement1 month post vaccination

Pro-inflammatory cytokine (IL-12) and anti-inflammatory cytokine (IL-10) levels will be evaluated in serum and in IL-1 stimulated whole blood supernatants using an ELISA assay.

Intracellular HO-1 expression in monocytes1 month post vaccination

Intracellular monocyte heme oxidase-1 (HO-1) expression will be measured by flow cytometry.The protein expression of HO-1 will be confirmed by Western blot. A commercial ELISA kit will be used in parallel to assess HO-1 levels in PBMC cell lysate.

HO-1 level in serum1 month post vaccination

Monocyte heme oxidase-1 (HO-1) level in serum will be measured by a commercial ELISA kit

Immune response to vaccination1 month post vaccination

Post-vaccination serum H1N1 antibodies titers (IgG and IgM) will be measured by an ELISA kit

Identification of T regulatory cells1 month post vaccination

Evaluation of Treg cells in peripheral blood mononuclear cells (PBMC) will be performed by flow cytometry using appropriate fluorochrome conjugated monoclonal antibodies for CD25 and FoxP3 markers

Secondary Outcome Measures
NameTimeMethod
Identification of T regulatory cells6 months post vaccination

Evaluation of Treg cells in PBMC will be performed by flow cytometry using appropriate fluorochrome conjugated monoclonal antibodies for CD25 and FoxP3 markers

Intracellular HO-1 expression in monocytes6 months post vaccination

Intracellular HO-1 expression will be measured by flow cytometry.The protein expression of HO-1 will be confirmed by Western blot. A commercial ELISA kit will be used in parallel to assess HO-1 levels in PBMC cell lysate.

HO-1 level in serum6 months post vaccination

HO-1 level in serum will be measured by a commercial ELISA kit

Immune response to vaccination6 months post vaccination

Post-vaccination serum H1N1 antibodies titers (IgG and IgM) will be measured by an ELISA kit

Cytokines levels measurement6 months post vaccination

Pro-inflammatory cytokine (IL-12) and anti-inflammatory cytokine (IL-10) levels will be evaluated in serum and in IL-1 stimulated whole blood supernatants using an ELISA assay.

Trial Locations

Locations (2)

CHU Brugmann

🇧🇪

Brussels, Belgium

HUDERF

🇧🇪

Brussels, Belgium

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