Nattiya Teawtrakul, Duantida Songdej, Chattree Hantaweepant, Adisak Tantiworawit, Sasinee Hantrakool, Pornpun Sripornsawan, Pacharapan Surapolchai, Supanun Lauhasurayotin, Kitti Torcharus, Pranee Sutcharitchan, Noppacharn Uaprasert, Krissana Panrong, Napat Laoaroon, Patcharee Komvilaisak, Panachai Silpsamrit, Arunotai Meekaewkunchorn, Bunchoo Pongtanakul, Pimlak Charoenkwan & Red Blood Cell Disorders Study Group
Srinagarind Hospital and Khon Kaen University. Ramathibodi Hospital, Siriraj Hospital and Mahidol University. Chiang Mai University. Prince of Songkla University. Thammasat University. Chulalongkorn University and King Chulalongkorn Memorial Hospital. Phramongkutklao College of Medicine. Khon Kaen University. Queen Sirikit National Institute of Child Health.
Thailand
Scientific Reports
Sci Rep 2026;
DOI: 10.1038/s41598-026-56773-2
Abstract
Hemoglobin H (Hb H) disease is a prevalent hereditary hemolytic anemia worldwide. Clinical severity varies by genotype, particularly between deletional and non-deletional forms, as well as the presence of co-inherited Hb E. However, disease-related complications remain underreported. We analyzed a Thai multicenter registry of 1,298 patients with Hb H disease to evaluate disease-related complications and risk factors. Deletional Hb H disease accounted for 55.9% of cases, whereas 44.1% had non-deletional disease. Hb E co-inheritance was present in 253 patients (19.5%). Patients with non-deletional Hb H disease and co-inherited Hb E had lower hemoglobin, higher ferritin levels, and greater transfusion requirements. Gallstones, pulmonary hypertension, extramedullary hematopoiesis, osteoporosis, and fractures were more frequent in non-deletional Hb H disease. After adjustment, increasing age (AOR 1.5; 95% CI 1.3-1.6; p<0.001), non-deletional Hb H disease (AOR 2.3; 95% CI 1.5-3.5; p<0.001), and transfusion dependency (AOR 2.7; 95% CI 1.4-5.3; p=0.003) were independently associated with overall complications. Splenectomy showed a marginal association (AOR 2.0; 95% CI 0.9-4.2; p=0.06). Gallstones were independently associated with non-deletional Hb H disease, splenectomy, and higher ferritin levels. Hyperuricemia was independently associated with increasing age and higher hemoglobin levels. These findings support genotype-informed monitoring in Hb H disease.
Category
Class V. Pulmonary Hypertension Associated with Hematological, Systemic, Metabolic, Nutritional and Other Disorders
|Symptoms and Findings Associated with Pulmonary Vascular Disease
Age Focus: Pediatric Pulmonary Vascular Disease or Adult Pulmonary Vascular Disease
Fresh or Filed Publication: Fresh (PHresh). Less than 1-2 years since publication
Article Access
Free PDF File or Full Text Article Available Through PubMed or DOI: Yes