Practical guidance for the diagnosis and management of secondary hypogammaglobulinemia: A Work Group Report of the AAAAI Primary Immunodeficiency and Altered Immune Response Committees

Iris M. Otani, Heather K. Lehman, Artemio M. Jongco, Lulu R. Tsao, Antoine E. Azar, Teresa K. Tarrant, Elissa Engel, Jolan E. Walter, Tho Q. Truong, David A. Khan, Mark Ballow, Charlotte Cunningham-Rundles, Huifang Lu, Mildred Kwan, Sara Barmettler

Research output: Contribution to journalArticlepeer-review

1 Scopus citations

Abstract

Secondary hypogammaglobulinemia (SHG) is characterized by reduced immunoglobulin levels due to acquired causes of decreased antibody production or increased antibody loss. Clarification regarding whether the hypogammaglobulinemia is secondary or primary is important because this has implications for evaluation and management. Prior receipt of immunosuppressive medications and/or presence of conditions associated with SHG development, including protein loss syndromes, are histories that raise suspicion for SHG. In patients with these histories, a thorough investigation of potential etiologies of SHG reviewed in this report is needed to devise an effective treatment plan focused on removal of iatrogenic causes (eg, discontinuation of an offending drug) or treatment of the underlying condition (eg, management of nephrotic syndrome). When iatrogenic causes cannot be removed or underlying conditions cannot be reversed, therapeutic options are not clearly delineated but include heightened monitoring for clinical infections, supportive antimicrobials, and in some cases, immunoglobulin replacement therapy. This report serves to summarize the existing literature regarding immunosuppressive medications and populations (autoimmune, neurologic, hematologic/oncologic, pulmonary, posttransplant, protein-losing) associated with SHG and highlights key areas for future investigation.

Original languageEnglish (US)
Pages (from-to)1525-1560
Number of pages36
JournalJournal of Allergy and Clinical Immunology
Volume149
Issue number5
DOIs
StatePublished - May 2022

Keywords

  • autoimmunity
  • B-cell–targeted therapy
  • CD19 CAR-T-cell therapy
  • immunosuppression
  • immunosuppressive medication
  • ocrelizumab
  • protein loss
  • protein-losing enteropathy
  • rituximab
  • Secondary hypogammaglobulinemia
  • solid organ transplant

ASJC Scopus subject areas

  • Immunology and Allergy
  • Immunology

Fingerprint

Dive into the research topics of 'Practical guidance for the diagnosis and management of secondary hypogammaglobulinemia: A Work Group Report of the AAAAI Primary Immunodeficiency and Altered Immune Response Committees'. Together they form a unique fingerprint.

Cite this