Low immunoglobulin M memory B-cell percentage in patients with heterotaxy syndrome correlates with the risk of severe bacterial infection

• Published in: Pediatric research Vol. 79; no. 2; pp. 271 - 277
• Main Authors: Chiu, Shuenn-Nan, Shao, Pei-Lan, Wang, Jou-Kou, Hsu, Hui-Wen, Lin, Ming-Tai, Chang, Luan-Yin, Lu, Chun-Yi, Lee, Ping-Ing, Huang, Li-Min, Wu, Mei-Hwan
• Format: Journal Article
• Language: English
• Published: New York Nature Publishing Group US 01.02.2016; Nature Publishing Group
• Subjects: 692/499; 692/53/2423; Adolescent; B-Lymphocytes - immunology; Bacterial infections; Bacterial Infections - diagnosis; Bacterial Infections - immunology; Bacterial Infections - microbiology; Cardiology; Case-Control Studies; Child; Child, Preschool; Congenital diseases; Female; Flow Cytometry; Heterotaxy Syndrome - complications; Heterotaxy Syndrome - diagnosis; Heterotaxy Syndrome - immunology; Humans; Immunocompromised Host; Immunoglobulin M - immunology; Immunoglobulins; Immunologic Memory; Immunophenotyping - methods; Infant; Lymphocyte Count; Male; Medicine; Medicine & Public Health; Opportunistic Infections - diagnosis; Opportunistic Infections - immunology; Opportunistic Infections - microbiology; Pediatric Surgery; Pediatrics; Phenotype; Risk Assessment; Risk Factors; Severity of Illness Index; Tertiary Care Centers; translational-investigation
• DOI: 10.1038/pr.2015.221
• ISSN: 0031-3998

Background: Patients with heterotaxy syndrome, commonly associated with complex congenital heart disease (CHD), exhibit a higher risk of severe bacterial infection (SBI). We sought to define the change of a novel immunologic marker, the immunoglobulin M (IgM) memory B-cell percentage, and its association with SBI. Methods: We enrolled 46 (M/F 29/17) heterotaxy syndrome patients (42 right atrial isomerism (RAI) and 4 left atrial isomerism (LAI)) aged > 1 y during the period 2010–2012 in a tertiary care center. We analyzed IgM + CD27 + memory B-cell percentages. Patients with simple and complex CHD served as controls. Results: The mean IgM memory B-cell percentages were the lowest in the heterotaxy syndrome group, compared with those in complex and simple CHD groups (1.8 ± 2.1 vs. 3.9 ± 3.2 vs. 5.1 ± 4.7, P < 0.001). In the heterotaxy syndrome group, 41.3% had low IgM memory B-cell percentages (<1% of B cells). Seven had a history of community-acquired SBI and 85.7% of these had low IgM memory B-cell percentages, which was the only significant factors related to community-acquired SBI ( P = 0.028). Conclusion: The memory B cell and IgM memory B-cell percentages are low in patients with heterotaxy syndrome, and the presence of IgM memory B-cell percentage < 1% correlates with community-acquired SBI.