Trough Concentrations of Specific Antibodies in Primary Immunodeficiency Patients Receiving Intravenous Immunoglobulin Replacement Therapy.

Ori Hassin,Arnon Broides,Amit Nahum,Yahya Abu Freih,Keren S Zrihen,Raz Somech,Atar Lev,Ran Hazan

doi:10.3390/jcm10040592

Abstract

Immunoglobulin replacement therapy is a mainstay therapy for patients with primary immunodeficiency (PID). The content of these preparations was studied extensively. Nevertheless, data regarding the effective specific antibodies content (especially in the nadir period), and, in different groups of PID patients is limited. We studied trough IgG concentrations as well as anti-Pneumococcus, anti-Haemophilus influenzae b, anti-Tetanus, and anti-Measles antibody concentrations in 17 PID patients receiving intravenous immunoglobulin (IVIg) compared with healthy controls matched for age and ethnicity. We also analyzed these results according to the specific PID diagnosis: X-linked agammaglobulinemia (XLA), combined immunodeficiency (CID), and ataxia telangiectasia (AT). We recorded a higher concentration of anti-pneumococcal polysaccharide antibodies in healthy controls compared to the entire group of PID patients. We also found significantly higher anti-tetanus toxoid antibody concentrations in the XLA patients, compared to CID patients. Anti-Haemophilus Influenzae b antibody titers were overall similar between all the groups. Interestingly, there were overall low titers of anti-Measles antibodies below protective cutoff antibody concentrations in most patients as well as in healthy controls. We conclude that relying on total IgG trough levels is not necessarily a reflection of effective specific antibodies in the patient’s serum. This is especially relevant to CID patients who may have production of nonspecific antibodies. In such patients, a higher target trough IgG concentration should be considered. Another aspect worth considering is that the use of plasma from adult donors with a waning immunity for certain pathogens probably affects the concentrations of specific antibodies in IVIg preparations.

Highlights

This article is an open access articleIntravenous immunoglobulins (IVIg) replacement is a mainstay therapy for patients with primary immunodeficiency, and for patients with defects in B or combined B and T cell dysfunction
Many studies have focused on the content of intravenous immunoglobulin (IVIg) preparations and most of them found no major difference between preparations and adequate concentrations of antibodies in commercial
We enrolled a total of 17 patients with primary immunodeficiency treated with IVIg in our institute and 17 healthy control subjects

Summary

Introduction

This article is an open access articleIntravenous immunoglobulins (IVIg) replacement is a mainstay therapy for patients with primary immunodeficiency, and for patients with defects in B or combined B and T cell dysfunction. Lack the ability to respond to specific stimuli, comprise the majority of patients in need for IVIg worldwide [1] Another significant group of patients requiring immunoglobulin replacement are patients with combined immunodeficiency, such as patients with what can be considered as “hyper IgE syndromes” (DOCK8 deficiency, STAT3 deficiency, IL6 receptor deficiency), hyper IgM syndrome, ataxia telangiectasia patients, and “leaky SCIDs”. The preparation of IVIg is regulated and agencies such as the World health Organization (WHO) have issued guidelines for manufacturing, including a minimum number of blood donor plasma to be included in each batch This was decided in order to give the patients a wide variety of antibodies, protecting against as many pathogens as possible, with a wide repertoire of antibodies [7]. Many studies have focused on the content of IVIg preparations and most of them found no major difference between preparations and adequate concentrations of antibodies in commercial

Methods

Results

Conclusion