Lymphocyte Immunophenotyping in Common Variable Immunodeficiency

The safety and scientific validity of this study is the responsibility of the study sponsor and investigators. Listing a study does not mean it has been evaluated by the U.S. Federal Government. Read our disclaimer for details. 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
Information provided by:
Barts & The London NHS Trust

September 6, 2010
September 8, 2010
September 8, 2010
March 2010
June 2010   (Final data collection date for primary outcome measure)
  • 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.
  • 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.
Same as current
No Changes Posted
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.
Same as current
Not Provided
Not Provided
Lymphocyte Immunophenotyping in Common Variable Immunodeficiency
Investigation of the Lymphocyte Surface Expression of Patients With Primary Immunodeficiency (Common Variable Immunodeficiency (CVID)), Compared to Controls

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.

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.
Observational Model: Case Control
Time Perspective: Cross-Sectional
Not Provided
Retention:   Samples With DNA
Blood serum samples kept for one year in secure laboratory.
Non-Probability Sample
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.
  • Common Variable Immunodeficiency
  • Granulomatous Disease
  • Bronchiectasis
  • Immunoglobulin Treatment
Not Provided
  • 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
Wehr C, Kivioja T, Schmitt C, Ferry B, Witte T, Eren E, Vlkova M, Hernandez M, Detkova D, Bos PR, Poerksen G, von Bernuth H, Baumann U, Goldacker S, Gutenberger S, Schlesier M, Bergeron-van der Cruyssen F, Le Garff M, Debré P, Jacobs R, Jones J, Bateman E, Litzman J, van Hagen PM, Plebani A, Schmidt RE, Thon V, Quinti I, Espanol T, Webster AD, Chapel H, Vihinen M, Oksenhendler E, Peter HH, Warnatz K. The EUROclass trial: defining subgroups in common variable immunodeficiency. Blood. 2008 Jan 1;111(1):77-85. Epub 2007 Sep 26.

*   Includes publications given by the data provider as well as publications identified by Identifier (NCT Number) in Medline.
Unknown status
Same as current
July 2011
June 2010   (Final data collection date for primary outcome measure)

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)/
Sexes Eligible for Study: All
18 Years and older   (Adult, Older Adult)
Contact information is only displayed when the study is recruiting subjects
United Kingdom
Not Provided
Not Provided
Hilary Longhurst, Barts and the London NHS Trust
Barts & The London NHS Trust
Not Provided
Principal Investigator: Hilary Longhurst, MBBS, PhD Barts and the London NHS Trust
Barts & The London NHS Trust
September 2010