Clinical trial NCT01196702: Lymphocyte Immunophenotyping in Common Variable Immunodeficiency
Official title: Investigation of the Lymphocyte Surface Expression of Patients With Primary Immunodeficiency (Common Variable Immunodeficiency (CVID)), Compared to Controls
Study type: OBSERVATIONAL
Overall status: COMPLETED
Conditions: Common Variable Immunodeficiency
Granulomatous Disease
Bronchiectasis
Immunoglobulin Treatment
Keywords: common variable immunodeficiency
granulomatous disease
crohns disease
bronchiectasis
immunoglobulin replacement or treatment
B-cell immunophenotyping
Lead sponsor: Barts & The London NHS Trust
Sponsor class: OTHER
First posted: 2010-09-08
Enrollment: 210
Sex: ALL
Minimum age (years): 18
Standard ages: ADULT
OLDER_ADULT
Accepts healthy volunteers: yes
Observational model: CASE_CONTROL
Time perspective: CROSS_SECTIONAL
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.

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.

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.

Eligibility 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)/

Primary outcomes:
measure: Percentage of B-cells of all lymphocytes
timeFrame: 5 months
measure: Percentage of class switched memory B-cells as a percentage of B-cells
timeFrame: 5 months
Secondary outcomes:
measure: Percentage expression of CD21 and CD38
timeFrame: 5 months
Other outcomes:
Locations:
city: London
country: United Kingdom
facility: Barts and the London NHS Trust
Study officials:
References:
citation: 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, Debre 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. doi: 10.1182/blood-2007-06-091744. Epub 2007 Sep 26.