Clinical Trial: Immune Regulation in Patients With Common Variable Immunodeficiency and Related Syndromes

Study Status: Recruiting
Recruit Status: Recruiting
Study Type: Observational

Official Title: Studies of Immune Regulation in Patients With Common Variable Immunodeficiency and Related Humoral Immunodeficiency Syndromes

Brief Summary:

This study will explore the cause of immunodeficiency in common variable immunodeficiency (CVI) and other related immunodeficiency syndromes IgA deficiency, hyper IgM syndrome, thymoma and agammaglobulinemia, hypogammaglobulinemia associated with Epstein-Barr infection, and others to better focus on how to correct the underlying defect.

Patients with CVI and their family members may participate in this study. Family members must be between the ages of 18 and 85, in good health and weigh at least 110 pounds.

Patients will receive standard medical care for their illness. Procedures may include a medical history and physical examination, routine blood tests, stool examination for infectious agents, lung function tests, chest and sinus X-rays. Treatment may include administration of immune serum globulin, antibiotics for infections, and anti-inflammatory drugs, if needed. In addition, patients may undergo the following:

  • Lymphapheresis: This procedure is done to collect large numbers of white blood cells (lymphocytes). Blood is collected through a needle in an arm vein, similar to donating blood. The blood is separated it into its components by centrifugation (spinning), the white cells are removed, and the rest of the blood (red cells, plasma and platelets) is returned to the body, either through the same needle or through another needle in the other arm.
  • Blood draw: Blood may be drawn through a needle in an arm vein (venipuncture). No more than 450 milliliters (15 ounces) of blood will be collected over a 6-week period from adults, and no more than 7 ml (1 1/2 teaspoons) per kilogram (2.2 pounds) of body weight in children over the same time period.
  • Lymph node biopsies: Lymph

    Detailed Summary:

    The purpose of this protocol is to carry out laboratory studies concerning the immunopathogenesis of Common Variable Immunodeficiency (CVI) and related primary humoral immunodeficiency diseases. Additionally, we aim to document and track the progression of known complications of this primary immunodeficiency. Complications associated with CVID include recurrent respiratory, and gastrointestinal bacterial infections, pulmonary insufficiency, nodular regenerative hyperplasia, lymphoid malignancy, and various autoimmune manifestations.

    Patients with CVI and related B Cell immunodeficiencies will be enrolled into this natural history study. Protocol procedures will include baseline measurements of and changes in lab and radiographic studies. Changes in the patients clinical state will be measured to determine the precursors of disease complications. This may lead to developments in improving preventive measures and novel treatment options for this population.


    Sponsor: National Institute of Allergy and Infectious Diseases (NIAID)

    Current Primary Outcome: No set outcome - evaluation of the course of the disease [ Time Frame: Ongoing ]

    Original Primary Outcome:

    Current Secondary Outcome:

    Original Secondary Outcome:

    Information By: National Institutes of Health Clinical Center (CC)

    Dates:
    Date Received: November 3, 1999
    Date Started: September 14, 1989
    Date Completion:
    Last Updated: April 21, 2017
    Last Verified: November 21, 2016