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Objective <p>To compare the clinical, laboratory, and treatment characteristics of classical, common variable immunodeficiency (CVID)-related, and syndromic agammaglobulinemia in children with profound hypogammaglobulinemia.</p> Methods <p>We retrospectively reviewed 25 children with profound hypogammaglobulinemia evaluated between 2005 and 2023. Patients were classified as classical (G1), CVID-related (G2a), or syndromic (G2b) agammaglobulinemia, and their clinical presentation, laboratory findings, and treatment outcomes were compared.</p> Results <p>Of the 25 children, 20 (80%) were boys. Classical agammaglobulinemia was diagnosed at a younger age and was associated with a longer duration of immunoglobulin replacement therapy. A family history of unexplained deaths was more frequent in non-classical forms. Syndromic agammaglobulinemia was characterized by growth failure and lower platelet counts.</p> Conclusion <p>Agammaglobulinemia comprises clinically distinct classical, CVID-related, and syndromic forms. Recognition of phenotypic clues, including family history, growth failure, and thrombocytopenia, may facilitate earlier diagnosis, timely referral, and initiation of immunoglobulin replacement therapy, thereby reducing infection-related morbidity.</p>

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Clinical and Laboratory Features of Pediatric Agammaglobulinemia: A Comparative Analysis of Classical, Common Variable Immunodeficiency, and Syndromic Forms

  • 期刊论文

  • 发表日期：

    2026年7月23日

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  • Tugce Akkus,
  • Mehmet Ali Karaselek,
  • Sukru Nail Guner,
  • Sevgi Keles,
  • Ismail Reisli

摘要

Objective

To compare the clinical, laboratory, and treatment characteristics of classical, common variable immunodeficiency (CVID)-related, and syndromic agammaglobulinemia in children with profound hypogammaglobulinemia.

Methods

We retrospectively reviewed 25 children with profound hypogammaglobulinemia evaluated between 2005 and 2023. Patients were classified as classical (G1), CVID-related (G2a), or syndromic (G2b) agammaglobulinemia, and their clinical presentation, laboratory findings, and treatment outcomes were compared.

Results

Of the 25 children, 20 (80%) were boys. Classical agammaglobulinemia was diagnosed at a younger age and was associated with a longer duration of immunoglobulin replacement therapy. A family history of unexplained deaths was more frequent in non-classical forms. Syndromic agammaglobulinemia was characterized by growth failure and lower platelet counts.

Conclusion

Agammaglobulinemia comprises clinically distinct classical, CVID-related, and syndromic forms. Recognition of phenotypic clues, including family history, growth failure, and thrombocytopenia, may facilitate earlier diagnosis, timely referral, and initiation of immunoglobulin replacement therapy, thereby reducing infection-related morbidity.