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Hemoglobin H (HbH) disease is associated with anemia, ineffective erythropoiesis, and iron overload. We report a case of a patient with HbH/Hb Constant Spring disease, who was maintained on chronic transfusions as an adult due to symptomatic anemia. Over time, he developed iron overload and was started on chelation therapy but did not have an adequate response to chelation.
View Article and Find Full Text PDFFolate metabolism in myelofibrosis: a missing key?
Ann Hematol
January 2025
Department of Medicine and Surgery, Anatomy Unit, University of Parma, Via Gramsci 14, Parma, 43126, Italy.
Folates serve as key enzyme cofactors in several biological processes. Folic acid supplementation is a cornerstone practice but may have a "dark side". Indeed, the accumulation of circulating unmetabolized folic acid (UMFA) has been associated with various chronic inflammatory conditions, including cancer.
View Article and Find Full Text PDFArticle Synopsis
- Thalassaemia stems from over 250 mutations in the beta globin gene, impacting hematopoietic stem cell differentiation and causing ineffective red blood cell production.
- The traditional focus on managing symptoms with transfusions and iron chelation therapy has hindered progress toward developing cell-based treatments, despite advancements in understanding the disease since the identification of the beta039 mutation in 1979.
- Recent progress in treating hematopoietic stem cell disorders emphasizes a 'target cell strategy,' suggesting a shift toward innovative treatments for thalassaemia that identify suitable candidates through risk stratification, highlighting its nature as a congenital HSC disorder.
Phosphorus-independent role of FGF23 in erythropoiesis and iron homeostasis.
PLoS One
December 2024
Department of Molecular Pathobiology, New York University College of Dentistry, New York, NY, United States of America.
A number of studies have reported an association between phosphorus, red blood cell (RBC) production, and iron metabolism. However, it is difficult to distinguish whether the effect of phosphorus is direct or through the actions of FGF23, and it is not clear whether phosphorus is positively or negatively associated with RBC production. In the present study, we investigated the effects of a) increased phosphorus load and b) phosphorus deficiency on erythropoiesis and iron metabolism in association with FGF23.
View Article and Find Full Text PDFThe role of miR-129-5p in regulating γ-globin expression and erythropoiesis in β-thalassemia.
Hum Mol Genet
December 2024
College of Clinical Medicine for Obstetrics & Gynecology and Pediatrics, Fujian Medical University, 88 Jiaotong Road, Taijiang District, Fuzhou 350004, China.
The regulation of γ-globin expression is crucial due to its beneficial effects on diseases like β-thalassemia and sickle cell disease. B-cell lymphoma/leukemia 11A (BCL11A) is a significant suppressor of γ-globin, and microRNAs (miRNAs) targeting BCL11A have been shown to alleviate this suppression. In our previous high-throughput sequencing, we identified an 11.
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