TY - JOUR
T1 - Practical guidance for the diagnosis and management of secondary hypogammaglobulinemia
T2 - A Work Group Report of the AAAAI Primary Immunodeficiency and Altered Immune Response Committees
AU - Otani, Iris M.
AU - Lehman, Heather K.
AU - Jongco, Artemio M.
AU - Tsao, Lulu R.
AU - Azar, Antoine E.
AU - Tarrant, Teresa K.
AU - Engel, Elissa
AU - Walter, Jolan E.
AU - Truong, Tho Q.
AU - Khan, David A.
AU - Ballow, Mark
AU - Cunningham-Rundles, Charlotte
AU - Lu, Huifang
AU - Kwan, Mildred
AU - Barmettler, Sara
N1 - Funding Information:
Disclosure of potential conflict of interest: H. K. Lehman is a clinical trial site principal investigator for Kedrion and Leadiant Biosciences, and a consultant and clinical trial site principal investigator for Chiesi Pharmaceutical S.p. A. A. Jongco has investigator-initiated grants from Takeda and Horizon Therapeutics. A. Azar has received funding from Grifols and X4 Pharmaceuticals. J. E. Walter has received funding from Takeda, Janssen, Chiesi, MustangBio, ADMA Biologicals, and Octapharma and is on consultant/advisory boards for Takeda, X4-Pharmaceuticals, CLS-Behring, Grifols, ADMA Biologicals, Enzyvant, and Regeneron. M. Ballow is a consultant or speaker for Alladapt DSMB, Grifols, Immune Deficiency Foundation, USIDNET, IgNS, UpToDate, Green Cross DSMB, and Syneos Health. S. Barmettler is supported by the National Institute of Allergy and Infectious Diseases of the National Institutes of Health under award number K23AI163350. The content is solely the responsibility of the authors and does not necessarily represent the official views of the National Institutes of Health. None of these pose a conflict of interest regarding this article. The rest of the authors declare that they have no relevant conflict of interest.
Funding Information:
No funding was received specifically for this work. Disclosure of potential conflict of interest: H. K. Lehman is a clinical trial site principal investigator for Kedrion and Leadiant Biosciences, and a consultant and clinical trial site principal investigator for Chiesi Pharmaceutical S.p. A. A. Jongco has investigator-initiated grants from Takeda and Horizon Therapeutics. A. Azar has received funding from Grifols and X4 Pharmaceuticals. J. E. Walter has received funding from Takeda, Janssen, Chiesi, MustangBio, ADMA Biologicals, and Octapharma and is on consultant/advisory boards for Takeda, X4-Pharmaceuticals, CLS-Behring, Grifols, ADMA Biologicals, Enzyvant, and Regeneron. M. Ballow is a consultant or speaker for Alladapt DSMB, Grifols, Immune Deficiency Foundation, USIDNET, IgNS, UpToDate, Green Cross DSMB, and Syneos Health. S. Barmettler is supported by the National Institute of Allergy and Infectious Diseases of the National Institutes of Health under award number K23AI163350. The content is solely the responsibility of the authors and does not necessarily represent the official views of the National Institutes of Health. None of these pose a conflict of interest regarding this article. The rest of the authors declare that they have no relevant conflict of interest.
Publisher Copyright:
© 2022 The Authors
PY - 2022/5
Y1 - 2022/5
N2 - 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.
AB - 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.
KW - B-cell–targeted therapy
KW - CD19 CAR-T-cell therapy
KW - Secondary hypogammaglobulinemia
KW - autoimmunity
KW - immunosuppression
KW - immunosuppressive medication
KW - ocrelizumab
KW - protein loss
KW - protein-losing enteropathy
KW - rituximab
KW - solid organ transplant
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U2 - 10.1016/j.jaci.2022.01.025
DO - 10.1016/j.jaci.2022.01.025
M3 - Article
C2 - 35176351
AN - SCOPUS:85127308302
SN - 0091-6749
VL - 149
SP - 1525
EP - 1560
JO - Journal of Allergy and Clinical Immunology
JF - Journal of Allergy and Clinical Immunology
IS - 5
ER -