Safe Subcutaneous Immunoglobulin Replacement Therapy in the Treatment of X-Linked Agammaglobulinemia Patient: A Case Report

Patuzzo Giuseppe, Tinazzi Elisa, Puccetti Antonio and Lunardi Claudio

Department of Autoimmune Diseases, University of Verona, Verona, Italy
Department of Immunology, Pediatric Hospital Bambino Gesù, Rome, Italy

^*Correspondance to: Patuzzo Giuseppe 

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Abstract:

Bruton’s agammaglobulinemia is a rare X-linked humoral immunodeficiency (XLA) characterized by recurrent bacterial infections. The usual treatment of this primary immunodeficiency consists of life-long immunoglobulins (Igs) replacement, administered intravenously or subcutaneously. We report the case of an18-year-oldpatient affected by XLA, diagnosed during childhood. Over time, his subcutaneous Igs dose had been progressively reduced and the intervals between administrations prolonged. After three years of subcutaneous Igs administration, he was able to maintain an IgG level higher than 700 mg/dl, by taking 10.000 mg of subcutaneous Igs every 20 days. This dose regimen provided a continuous protection against infections, while no significant adverse event was observed. Patient adherence was guaranty by a home-based therapy; a regular follow-up and serum IgG level monitoring were also assured. Given the shortage of Ig available for the treatment of different primary immunodeficiencies, a therapy that, with a low Ig amount together with a reduced number of administrations, is able tosustaining a safe IgG level, is essential to preserve patient adherence.

Keywords:

X-linked agammaglobulinemia; Bruton’s tyrosine kinase (Btk); Subcutaneous immunoglobulin (SCIG); Local tolerability; Serum IgG trough level

Cite the Article:

Giuseppe P, Elisa T, Antonio P, Claudio L. Safe Subcutaneous Immunoglobulin Replacement Therapy in the Treatment of X-Linked Agammaglobulinemia Patient: A Case Report. Ann Clin Case Rep. 2017; 2: 1333.