Early Subcutaneous Immunoglobulin for X-Linked Agammaglobulinemia

Alfredo Tagarro

doi:10.23937/2378-3672/1410016

Abstract

Introduction: Subcutaneous immunoglobulin (SCIG) utilization is limited in Spain. Our aim was to describe our experience with SCIG from the diagnosis in a patient with X-linked agammaglobulinemia (XLA). Case Presentation: A recently diagnosed 6-year-old child with XLA was started on SCIG. A loading dose of 6 g, in three consecutive days (2 g/day) was prescribed. Initially, the aim was trough serum IgG over 500 mg/dL. Nevertheless, 15 months later, IgG levels fell below 500 mg/dL, and some infections occurred. A new aim of IgG over 700 mg/dLwas established, after current recommendations. Dose was increased to 3 g every 7 days. IgG levels rose over 700 mg/dL, and infections disappeared. Parental evaluation of quality of life is good. No adverse events were reported. Conclusions: SCIG is a valuable choice for treating XLA patients, even from the very beginning.

Highlights

Subcutaneous immunoglobulin (SCIG) utilization is limited in Spain
SCIG is a valuable choice for treating X-linked agammaglobulinemia (XLA) patients, even from the very beginning
X-linked agammaglobulinemia (XLA) or Bruton agammaglobulinemia, is an inherited immunodeficiency disease caused by mutations in the gene BTK, that codes for Bruton tyrosine kinase (BTK)

Summary

Introduction

Subcutaneous immunoglobulin (SCIG) utilization is limited in Spain. Our aim was to describe our experience with SCIG from the diagnosis in a patient with X-linked agammaglobulinemia (XLA).Case Presentation: A recently diagnosed 6-year-old child with XLA was started on SCIG. Subcutaneous immunoglobulin (SCIG) utilization is limited in Spain. Our aim was to describe our experience with SCIG from the diagnosis in a patient with X-linked agammaglobulinemia (XLA). The aim was trough serum IgG over 500 mg/dL. A new aim of IgG over 700 mg/dLwas established, after current recommendations. IgG levels rose over 700 mg/dL, and infections disappeared. Without mature B lymphocytes, antibody-producing plasma cells are absent. XLA is characterized by low levels of serum immunoglobulins and almost total or total absence of B lymphocytes and plasma cells. The current management is the immunoglobulin replacement therapy (IRT). Utilization of SCIG it is still limited in Spain. The use of SCIG is sequential, after a loading dose IVIG

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