Abstract

Immunoglobulin (Ig) replacement therapy dramatically changed the clinical course of primary hypogammaglobulinemias, significantly reducing the incidence of infectious events. Over the last two decades its use has been extended to secondary antibody deficiencies, particularly those related to hematological disorders as lymphoproliferative diseases (LPDs) and multiple myeloma. In these malignancies, hypogammaglobulinemia can be an intrinsic aspect of the disease or follow chemo-immunotherapy regimens, including anti-CD20 treatment. Other than in LPDs the broadening use of immunotherapy (e.g., rituximab) and immune-suppressive therapy (steroids, sulfasalazine, and mycophenolate mofetil) has extended the occurrence of iatrogenic hypogammaglobulinemia. In particular, in both autoimmune diseases and solid organ transplantation Ig replacement therapy has been shown to reduce the rate of infectious events. Here, we review the existing literature about Ig replacement therapy in secondary hypogammaglobulinemia, with special regard for subcutaneous administration route, a safe, effective, and well-tolerated treatment approach, currently well established in primary immunodeficiencies and secondary hypogammaglobulinemias.

Highlights

  • Immunoglobulins (Ig) have been commercially available since the 1940s, and first carried out as replacement therapy (IgRT) in a patient with agammaglobulinemia in 1951 by Ogden Bruton

  • From that first experience on, Ig have been widely used in patients with primary immune deficiency (PID), as it is well known that hypogammaglobulinemia is a crucial risk factor for development of infectious events and IgRT is able to reduce this risk

  • The lack of clear indications regarding the use of prophylactic IVIG in secondary antibody deficiencies (SAD) may appear paradoxical, if we consider the broad range of disorders in which hypogammaglobulinemia is an intrinsic aspect of the disease or a iatrogenic consequence (Table 1) and being the number of affected patients significantly higher than in PID

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Summary

Immunoglobulin replacement therapy in secondary hypogammaglobulinemia

Over the last two decades its use has been extended to secondary antibody deficiencies, those related to hematological disorders as lymphoproliferative diseases (LPDs) and multiple myeloma In these malignancies, hypogammaglobulinemia can be an intrinsic aspect of the disease or follow chemo-immunotherapy regimens, including anti-CD20 treatment. Other than in LPDs the broadening use of immunotherapy (e.g., rituximab) and immune-suppressive therapy (steroids, sulfasalazine, and mycophenolate mofetil) has extended the occurrence of iatrogenic hypogammaglobulinemia. In both autoimmune diseases and solid organ transplantation Ig replacement therapy has been shown to reduce the rate of infectious events.

INTRODUCTION
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