Disease-related complications and risk factors in hemoglobin H disease in a Thai multicenter registry
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.
Authors
- Chattree Hantaweepant (ORCID: https://orcid.org/0000-0003-0996-3789)
- Panachai Silpsamrit (ORCID: https://orcid.org/0000-0002-2679-4254)
- Bunchoo Pongtanakul (ORCID: https://orcid.org/0000-0003-4204-0118)
- Hansamon Poparn (ORCID: https://orcid.org/0000-0001-9389-4653)
- Sasinee Hantrakool (ORCID: https://orcid.org/0000-0002-1248-9734)
- Noppacharn Uaprasert (ORCID: https://orcid.org/0000-0003-4562-9139)
- Pacharapan Surapolchai (ORCID: https://orcid.org/0000-0003-4180-8041)
- Supanun Lauhasurayotin (ORCID: https://orcid.org/0000-0002-2432-8022)
- Phakatip Sinlapamongkolkul (ORCID: https://orcid.org/0000-0001-9602-4768)
- Pimlak Charoenkwan (ORCID: https://orcid.org/0000-0002-9123-9177)
- Krissana Panrong
- Pranee Sutcharitchan
- Pornpun Sripornsawan (ORCID: https://orcid.org/0000-0001-5130-0714)
- Napat Laoaroon (ORCID: https://orcid.org/0000-0003-1517-9464)
- Kitti Torcharus
- Shevachut Chavananon (ORCID: https://orcid.org/0000-0003-0961-5063)
- Arunotai Meekaewkunchorn (ORCID: https://orcid.org/0000-0003-0336-0207)
- Nattiya Teawtrakul (ORCID: https://orcid.org/0000-0002-2364-1498)
- Adisak Tantiworawit (ORCID: https://orcid.org/0000-0002-2381-9292)
- Patcharee Komvilaisak
- Duantida Songdej
- Red Blood Cell Disorders Study Group
Institutions
- Thammasat University (TH)
- Prince of Songkla University (TH)
- Siriraj Hospital (TH)
- Chulalongkorn University (TH)
- Khon Kaen University (TH)
- Mahidol University (TH)
- King Chulalongkorn Memorial Hospital (TH)
- Queen Sirikit National Institute of Child Health (TH)
- Phramongkutklao Hospital (TH)
- Ramathibodi Hospital (TH)
- Phramongkutklao College of Medicine
- Chiang Mai University (TH)
Publication Details
- Journal
- Scientific Reports
- Published
- 2026-06-13
- DOI
- https://doi.org/10.1038/s41598-026-56773-2
- Primary Topic
- Hemoglobinopathies and Related Disorders
- Type
- article
- Field-Weighted Citation Impact
- 0.00