{"title":"Hemoglobin H Disease and Growth: A Comparative Study of DHbH and NDHbH Patients.","authors":"Issanun Hunnuan, Kleebsabai Sanpkit, Ornsuda Lertbannaphong, Jassada Buaboonnam","doi":"10.4084/MJHID.2023.045","DOIUrl":null,"url":null,"abstract":"<p><strong>Background: </strong>Hemoglobin H disease <i>(</i>HbH<i>)</i>, a hemoglobinopathy resulting from abnormal alpha globin genes, is classified into two categories: deletional HbH <i>(</i>DHbH<i>)</i> and non-deletional HbH <i>(</i>NDHbH<i>)</i>. The alpha-mutation genotypes exhibit a range of clinical anemias, which differentially impact patient growth.</p><p><strong>Objectives: </strong>This retrospective study assessed the growth of HbH patients at Siriraj Hospital, Mahidol University.</p><p><strong>Methods: </strong>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 BMI-for-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.</p><p><strong>Results: </strong>Of the 145 HbH patients, 75 <i>(</i>51.7<i>%)</i> had NDHbH, with --<sup>SEA</sup><i>/</i>α<sup>CS</sup>α being the most common genotype <i>(</i>70 patients; 93.3<i>%)</i>. The mean baseline hemoglobin level was significantly lower in NDHbH patients than in DHbH patients (8.16 ± 0.93 g<i>/</i>dL vs. 9.51 ± 0.68 g<i>/</i>dL; <i>P</i> < 0.001<i>)</i>. Splenomegaly and growth failure prevalences were higher in NDHbH patients (37.3<i>%</i> vs. 0%, with <i>P</i> < 0.001, and 22.7% vs. 8.6%, with <i>P</i> = 0.020, respectively). Multivariable analysis revealed splenomegaly > 3 cm was associated with growth failure (OR = 4.28; 95% CI, 1.19-15.39; <i>P</i> = 0.026).</p><p><strong>Conclusions: </strong>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.</p>","PeriodicalId":18498,"journal":{"name":"Mediterranean Journal of Hematology and Infectious Diseases","volume":null,"pages":null},"PeriodicalIF":2.0000,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/f1/4c/mjhid-15-1-e2023045.PMC10497309.pdf","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"Mediterranean Journal of Hematology and Infectious Diseases","FirstCategoryId":"3","ListUrlMain":"https://doi.org/10.4084/MJHID.2023.045","RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q3","JCRName":"HEMATOLOGY","Score":null,"Total":0}
引用次数: 0
Abstract
Background: Hemoglobin H disease (HbH), a hemoglobinopathy resulting from abnormal alpha globin genes, is classified into two categories: deletional HbH (DHbH) and non-deletional 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 BMI-for-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.
期刊介绍:
Reciprocal interdependence between infectious and hematologic diseases (malignant and non-malignant) is well known. This relationship is particularly evident in Mediterranean countries. Parasitosis as Malaria, Leishmaniosis, B Hookworms, Teniasis, very common in the southeast Mediterranean area, infect about a billion people and manifest prevalently with anemia so that they are usually diagnosed mostly by experienced hematologist on blood or bone marrow smear. On the other hand, infections are also a significant problem in patients affected by hematological malignancies. The blood is the primary vector of HIV infection, which otherwise manifest with symptoms related to a reduction in T lymphocytes. In turn, infections can favor the insurgency of hematological malignancies. The causative relationship between Epstein-Barr virus infection, Helicobacter pylori, hepatitis C virus, HIV and lymphoproliferative diseases is well known.