Indian pediatrics · 2026
Clinical and Laboratory Features of Pediatric Agammaglobulinemia: A Comparative Analysis of Classical, Common Variable Immunodeficiency, and Syndromic Forms.
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Akkus Tugce, Karaselek Mehmet Ali, Guner Sukru Nail, Keles Sevgi, Reisli Ismail
Аннотация
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.
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.