Hemoglobin H Disease and Growth: A Comparative Study of DHbH and NDHbH Patients
Issued Date
2023-01-01
Resource Type
eISSN
20353006
Scopus ID
2-s2.0-85174392813
Journal Title
Mediterranean Journal of Hematology and Infectious Diseases
Volume
15
Issue
1
Rights Holder(s)
SCOPUS
Bibliographic Citation
Mediterranean Journal of Hematology and Infectious Diseases Vol.15 No.1 (2023)
Suggested Citation
Hunnuan I., Sanpkit K., Lertbannaphong O., Buaboonnam J. Hemoglobin H Disease and Growth: A Comparative Study of DHbH and NDHbH Patients. Mediterranean Journal of Hematology and Infectious Diseases Vol.15 No.1 (2023). doi:10.4084/MJHID.2023.045 Retrieved from: https://repository.li.mahidol.ac.th/handle/123456789/90816
Title
Hemoglobin H Disease and Growth: A Comparative Study of DHbH and NDHbH Patients
Author(s)
Author's Affiliation
Other Contributor(s)
Abstract
Background: Hemoglobin H disease (HbH), a hemoglobinopathy resulting from abnormal alpha globin genes, is classified into two categories: deletional HbH (DHbH) and nondeletional HbH (NDHbH). The alpha-mutation genotypes exhibit a range of clinical anemias, which differentially impact patient growth. Objectives: This retrospective study assessed the growth of HbH patients at Siriraj Hospital, Mahidol University. Methods: Patients diagnosed with HbH between January 2005 and April 2021 were analyzed using growth standard scores of the Thai Society for Pediatric Endocrinology (2022 version) and BMIfor- age Z scores of the World Health Organization. Growth failure was defined as a patient’s height for age exceeding two standard deviations below the mean. Results: Of the 145 HbH patients, 75 (51.7%) had NDHbH, with -SEA/αCSα being the most common genotype (70 patients; 93.3%). The mean baseline hemoglobin level was significantly lower in NDHbH patients than in DHbH patients (8.16 ± 0.93 g/dL vs. 9.51 ± 0.68 g/dL; P < 0.001). Splenomegaly and growth failure prevalences were higher in NDHbH patients (37.3% vs. 0%, with P <0.001, and 22.7% vs. 8.6%, with P = 0.020, respectively). Multivariable analysis revealed splenomegaly > 3 cm was associated with growth failure (OR = 4.28; 95% CI, 1.19-15.39; P = 0.026). Conclusions: NDHbH patients exhibited lower hemoglobin levels and more pronounced splenomegaly than DHbH patients. Growth failure can occur in both HbH types but appears more prevalent in NDHbH. Close monitoring of growth velocity is essential, and early treatment interventions may be required to prevent growth failure.