Diagnostic Immunization with Bacteriophage ΦX 174 in Patients with Common Variable Immunodeficiency/Hypogammaglobulinemia.

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2014-01

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Abstract

Purpose

Use of the T cell-dependent neoantigen bacteriophage ΦX 174 has been described since the 1960s as a method to assess specific antibody response in patients with primary immunodeficiencies. We reviewed a cohort of patients at Duke University Medical Center who received immunization with bacteriophage and report the clinical utility and safety of the immunization, as well as patient characteristics.

Methods

A retrospective chart review was performed of all Duke Immunology Clinic patients (pediatric and adult) who received immunizations with bacteriophage, from 1976 to 2012. Subjects were selected for inclusion if their diagnosis at the time of bacteriophage was either presumed or confirmed common variable immunodeficiency (CVID), hypogammaglobulinemia, transient hypogammaglobulinemia, or antibody deficiency unspecified. Follow up post-immunization was also recorded.

Results

One hundred twenty-six patients were identified, 36 adults and 90 pediatric patients. Diagnoses prior to bacteriophage were CVID (n = 100), hypogammaglobulinemia (n = 23), and antibody deficiency (n = 3). Post-immunization diagnoses were CVID (n = 65), hypogammaglobulinemia (n = 19), unknown (n = 23), no primary immune deficiency (n = 10), and other primary immunodeficiency (n = 9). Seventy-five patients had abnormal bacteriophage results, 37 were normal, and 14 were borderline. There were 257 recorded administrations of the immunization. Information was available on adverse reactions for 171 administrations. Fourteen immunizations were associated with minor adverse events. Nineteen patients stopped their immunoglobulin replacement therapy based on reported normal responses to immunization.

Conclusion

Bacteriophage ΦX 174 immunization is a safe, well-tolerated, and clinically useful method to assess antibody response in patients with suspected antibody-mediated immunodeficiencies, particularly those who are on immunoglobulin replacement therapy at the time of immunization.

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bacteriophage ΦX 174, common variable immunodeficiency, hypogammaglobulinemia, primary immunodeficiency

Citation

Published Version (Please cite this version)

10.3389/fimmu.2014.00410

Publication Info

Smith, Lauren L, Rebecca Buckley and Patricia Lugar (2014). Diagnostic Immunization with Bacteriophage ΦX 174 in Patients with Common Variable Immunodeficiency/Hypogammaglobulinemia. Frontiers in immunology, 5(AUG). p. 410. 10.3389/fimmu.2014.00410 Retrieved from https://hdl.handle.net/10161/32138.

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Scholars@Duke

Buckley

Rebecca Hatcher Buckley

James Buren Sidbury Distinguished Professor Emeritus of Pediatrics, in the School of Medicine

The overall emphasis of Dr. Buckley's research is in human T,B and NK cell development and in aberrations in their development and regulation. The work involves three particular areas of investigation: 1) the cellular and molecular bases of genetically-determined human immunodeficiency diseases, 2) the use of bone marrow stem cells to cure genetically-determined immunodeficiency diseases, and 3) the use of human SCID bone marrow stem cell chimeras to study human thymic education, T and B cell ontogeny, tolerance induction and MHC restriction mechanisms. Methodology includes monoclonal antibody (mAb) analyses of lymphocyte phenotypes, a variety of T cell and natural killer (NK) cell functional assays, studies of thymic output by T cell receptor recombination excision circle measurement, studies of T cell diversity by spectratyping, studies of T cell longevity by telomere analysis and assessment of B cell differentiation and function. A unique resource available for her studies is the largest population of patients with genetically-determined immunodeficiency diseases in the U.S., which includes the largest population in the world of longterm SCID chimeras treated at a single center, some of whom have been studied and followed for more than 37 years. The administration of rigorously T cell depleted haploidentical bone marrow stem cells to SCID recipients without pre-transplant conditioning or post-transplant use of immunosuppressive drugs to prevent GVHD provides an unmanipulated system for studying human thymic education, T and B cell ontogeny, MHC restriction mechanisms and tolerance induction. Studies to identify mutations in patients with primary immunodeficiency are continuing, particularly in those with SCID.

Lugar

Patricia Lynne Lugar

Associate Professor of Medicine

I have a strong interest and research background in the study of dysregulated B lymphocytes, B cells, that characterize clinical disease such as autoimmune disease and humoral immune deficiency. My current research projects are aimed at the study of humoral immune deficiency, specifically common variable immune deficiency or CVID and the dysregulation of B cells in the generation of lymphoma and autoimmune disease.
Other ongoing research projects include characterizing the molecular and cellular events that cause a common disease, chronic idiopathic urticaria or CIU and rare disorders idiopathic anaphylaxis and idiopathic angioedema.


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