Fetal hemoglobin levels and morbidity in untransfused patients with β-thalassemia intermedia.
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To evaluate the association between fetal hemoglobin (HbF) levels and morbidity in β-thalassemia intermedia (TI), we analyzed data from 63 untransfused patients who had also never received HbF induction therapy. Patient records were reviewed for any history of 10 predefined morbidities. Laboratory measurements for markers of ineffective erythropoiesis were also obtained. The mean age of patients was 32.1 years, 47.6% were males, and the median HbF level was 37.2%. HbF levels correlated positively with total hemoglobin, yet negatively with growth differentiation factor-15 and non–transferrin-bound iron levels. Median HbF levels were significantly lower in patients with the majority of evaluated morbidities than in those without. There was a strong negative adjusted linear correlation between the HbF level and the total number of morbidities (R2 = 0.825, P < .001). The HbF threshold of 63.7% had 95.5% sensitivity and 100% specificity for ensuring absence of morbidity. There exists a strong association between HbF levels and morbidity in the subset of untransfused patients with TI.
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Higher fetal hemoglobin levels are linked to lower morbidity and mortality in TDT and SCD.
“Elevated levels of fetal hemoglobin (consisting of two alpha and two gamma chains) are associated with improved morbidity and mortality in patients with TDT and SCD.[20][21][22]”
From CRISPR-Cas9 Gene Editing for Sickle Cell Disease and β-Thalassemia (2021) · cites this paper as [20]
The Introduction reports that HbF modifies morbidity and mortality in adults with sickle cell disease. The Abstract and Results and discussion find lower morbidity with higher HbF in untransfused patients with β-thalassemia intermedia, not transfusion-dependent thalassemia (TDT); the study does not establish a mortality association in TDT. Passage-level page numbers are unavailable in our copy. (LLM, conf=0.88)
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References 23 · Cited by 103
Research works printed but not linked: 2
- [6]6. Galanello R, Sanna S, Perseu L, et al. Ameliora- tion of Sardinian beta0 thalassemia by genetic modifiers. Blood. 2009;114(18):3935-3937.
- [9]9. Rivella S. Ineffective erythropoiesis and thalasse- mias. Curr Opin Hematol. 2009;16(3):187-194.
- Authors
- Musallam, K. M., Sankaran, V. G., Cappellini, M. D., Duca, L., Nathan, D. G., & Taher, A. T.
- Affiliations
- Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico (IT)
- Boston Children's Hospital (US)
- American University of Beirut Medical Center (LB)
- Venue
- Blood
- Publisher
- American Society of Hematology
- Published
- 2012-01-12
- Volume
- 119
- Issue
- 2
- Pages
- 364-367
- ISSN
- 0006-4971 (ISSN-L)
- 1528-0020
- OpenAlex
- W2064631448
- Cited by
- 103
- References
- 23
- Citations by year
- 2026: 9 · 2025: 3 · 2024: 6 · 2023: 15 · 2022: 7 · 2021: 8 · 2020: 6 · 2019: 4 · 2018: 7 · 2017: 4 · 2016: 9 · 2015: 6 · 2014: 8 · 2013: 6 · 2012: 5
- FWCI
- 3.05
- Type
- journal article
- Language
- en
- Open access
- bronze
- doi.org
- Locations
- Blood (publishedVersion)
- PubMed (publishedVersion)
- Archivio Istituzionale della Ricerca (Universita Degli Studi Di Milano) (submittedVersion, other-oa)
- Topics
- Health Sciences › Medicine › Genetics › Hemoglobinopathies and Related Disorders
- Health Sciences › Medicine › Hematology › Iron Metabolism and Disorders
- Health Sciences › Medicine › Hematology › Blood groups and transfusion
- Keywords
- fetal hemoglobin, beta-thalassemia intermedia, morbidity, growth differentiation factor 15, non-transferrin-bound iron, ineffective erythropoiesis
- MeSH terms
- Adolescent, Adult, Aged, Child, Cross-Sectional Studies, Erythropoiesis, Erythropoietin, Female, Fetal Hemoglobin, Humans, Male, Middle Aged, Morbidity, Receptors, Transferrin, beta-Thalassemia, Young Adult
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