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Lymphocyte Immunophenotyping in Common Variable Immunodeficiency

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ClinicalTrials.gov Identifier: NCT01196702
Recruitment Status : Unknown
Verified September 2010 by Barts & The London NHS Trust.
Recruitment status was:  Recruiting
First Posted : September 8, 2010
Last Update Posted : September 8, 2010
Sponsor:
Information provided by:

Study Description
Brief Summary:

The purpose of this study is to discover if differences in the surface markers of B-cells (antibody producing cells of the immune system) in Common Variable Immune Deficiency (CVID) are related to CVID or its complications/treatment (e.g. bronchiectasis, granulomatous disease, immunoglobulin treatment).

The study hypothesis is that the altered B-cell surface markers are related to CVID, and not to the complications or treatment of CVID.


Condition or disease
Common Variable Immunodeficiency Granulomatous Disease Bronchiectasis Immunoglobulin Treatment

Detailed Description:
Common Variable Immune Deficiency (CVID) is a syndrome containing a spectrum of disorders which results in weakened immunity and recurrent infections. The ESID (European Society for Immunodeficiencies) CVID definition includes patients with marked decrease of IgG (at least 2 standard deviations below the mean for age). Patients must also have disease onset at an age over 2 years, absent isohaemagglutinins and/or response to vaccines and other defined causes of hypogammaglobulinaemia must be excluded. The Euroclass system of classifying CVID is the result of a European multicentre trial attempting to develop a consensus of two existing classification schemes of B-cell immunophenotyping. In this paper it was shown that B-cell immunophenotype correlated with coincidence of clinical sequelae and it suggested implementing this to further classify CVID to give prognostic and therapeutic information. However, it has not yet been shown that these alterations in B-cell immunophenotype are the result of CVID itself and not caused by the treatment or complications of CVID (e.g. immunoglobulin replacement therapy, granulomatous disease, bronchiectasis). The aim of this study is to show that alterations in B-cell immunophenotype are caused by CVID itself and not by its complications or treatment. The study will therefore compare CVID patients to suitable control patients with granulomatous disease, bronchiectasis and on long-term immunoglobulin therapy. A control group of normal people will also be included to ensure the assay can detect normality and to show differences between normal people and patients with CVID.

Study Design

Study Type : Observational
Estimated Enrollment : 210 participants
Observational Model: Case Control
Time Perspective: Cross-Sectional
Official Title: Investigation of the Lymphocyte Surface Expression of Patients With Primary Immunodeficiency (Common Variable Immunodeficiency (CVID)), Compared to Controls
Study Start Date : March 2010
Primary Completion Date : June 2010
Estimated Study Completion Date : July 2011


Groups and Cohorts

Group/Cohort
CVID
Patients with common variable immunodeficiency
CVID and granulomatous disease
Patients with CVID complicated with granulomatous inflammation
CVID and bronchiectasis
Patients with CVID complicated by bronchiectasis
Control on Immunoglobulin
Patients on immunoglobulin long-term who do not have an immunodeficiency
Control bronchiectasis
Controls with bronchiectasis not caused by a known immunodeficiency
Control with granulomatous disease
Control patients with Crohn's Disease as this is a disease that causes granulomatous inflammation.
Healthy Controls


Outcome Measures

Primary Outcome Measures :
  1. Percentage of B-cells of all lymphocytes [ Time Frame: 5 months ]
    Look at percentage of cells within the lymphocyte gate that express the B-cell marker CD19, and compare to healthy controls and non-healthy controls.

  2. Percentage of class switched memory B-cells as a percentage of B-cells [ Time Frame: 5 months ]
    Percentage of class-switched memory B-cells (expressing CD27 and CD19), that do not express IgM or IgD, as a percentage of B-cells. This is reduced in CVID and this will be compared between controls and the patients with CVID.


Secondary Outcome Measures :
  1. Percentage expression of CD21 and CD38 [ Time Frame: 5 months ]
    Look for abnormalities in the CVID group and compare to control groups in the numeber of B-cells expressing low levels of CD21 (CD21 lo), and high CD38.


Biospecimen Retention:   Samples With DNA
Blood serum samples kept for one year in secure laboratory.

Eligibility Criteria

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Ages Eligible for Study:   18 Years and older   (Adult, Senior)
Sexes Eligible for Study:   All
Accepts Healthy Volunteers:   Yes
Sampling Method:   Non-Probability Sample
Study Population
Adult patients selected from medical clinics in the order of attendance with common variable immunodeficiency, bronchiectasis, on long-term immunoglobulin treament and granulomatous disease. Must be able to give consent for testing of B-cell immunophenotype. Healthy control samples taken from colleagues.
Criteria

Inclusion Criteria:

  • 18 or over
  • Competent to consent
  • Have diagnosis of Common Variable Immunodeficiency, granulomatous disease, on long term immunoglobulin or bronchiectasis.

Exclusion Criteria:

  • Under 18
  • Unable to consent.
  • Medical problem that could alter B-cell immunophenotype (except for the diagnoses in the inclusion criteria)/
Contacts and Locations

Information from the National Library of Medicine

To learn more about this study, you or your doctor may contact the study research staff using the contact information provided by the sponsor.

Please refer to this study by its ClinicalTrials.gov identifier (NCT number): NCT01196702


Contacts
Contact: Philip Bright, MBBS 02032460282 philip.bright@bartsandthelondon.nhs.uk
Contact: Hilary Bright, MBBS 02032460282/5 hilary.longhurst@bartsandthelondon.nhs.uk

Locations
United Kingdom
Barts and the London NHS Trust Recruiting
London, United Kingdom, E1 1BB
Contact: Gerry Leonard    02078827260    gerry.leonard@bartsandthelondon.nhs.uk   
Principal Investigator: Hilary Longhurst, MBBS, PhD         
Sponsors and Collaborators
Barts & The London NHS Trust
Investigators
Principal Investigator: Hilary Longhurst, MBBS, PhD Barts and the London NHS Trust
More Information

Publications:
Responsible Party: Hilary Longhurst, Barts and the London NHS Trust
ClinicalTrials.gov Identifier: NCT01196702     History of Changes
Other Study ID Numbers: 006749
First Posted: September 8, 2010    Key Record Dates
Last Update Posted: September 8, 2010
Last Verified: September 2010

Keywords provided by Barts & The London NHS Trust:
common variable immunodeficiency
granulomatous disease
crohns disease
bronchiectasis
immunoglobulin replacement or treatment
B-cell immunophenotyping

Additional relevant MeSH terms:
Immunologic Deficiency Syndromes
Common Variable Immunodeficiency
Bronchiectasis
Granuloma
Immune System Diseases
Bronchial Diseases
Respiratory Tract Diseases
Lymphoproliferative Disorders
Lymphatic Diseases
Pathologic Processes
Immunoglobulins
Antibodies
Immunologic Factors
Physiological Effects of Drugs