Alternative therapies of manifest forms of β-thalassemia: stimulation of fetal hemoglobin synthesis
- Authors: Krasilnikova M.V.1, Karamjan N.A.1
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Affiliations:
- Dmitriy Rogachev National Medical Research Center of Pediatric Hematology, Oncology, Immunology
- Issue: Vol 16, No 4 (2017)
- Pages: 90-97
- Section: Статьи
- Submitted: 09.08.2018
- Published: 09.11.2017
- URL: https://hemoncim.com/jour/article/view/14
- DOI: https://doi.org/10.24287/1726-1708-2017-16-4-90-97
- ID: 14
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Abstract
An imbalance in the synthesis and ratio of α and non-α globin chains is the major pathophysiological mechanism leading to ineffective erythropoiesis and hemolysis in severe form of the β-thalassemia. Phenotype in patients who retain the capacity to synthesize fetal hemoglobin (HbF) is milder due to decreasing α- /non-α globin chain imbalance. Induction of HbF might be an effective therapeutic strategy for severe form of β-thalassemia. This literature review presents data about efficiency safeness of different medication and their combination, which stimulate synthesis of HbF. This review can provide a new insight into the current status and future perspectives in HbF reactivation as treatment option for severe form of β-thalassemia.
About the authors
M. V. Krasilnikova
Dmitriy Rogachev National Medical Research Center of Pediatric Hematology, Oncology, Immunology
Email: marina.krasilnikova@fcchomoscow.ru
ORCID iD: 0000-0002-0929-4792
MD, hematologist, Department of Hematology, Institute of Hematology, Immunology and Cell Technology
Russia 117997, Moscow, Samory Mashela st., 1
Russian Federation
N. A. Karamjan
Dmitriy Rogachev National Medical Research Center of Pediatric Hematology, Oncology, Immunology
Author for correspondence.
Email: marina.krasilnikova@fcchomoscow.ru
ORCID iD: 0000-0002-9865-527X
Russian Federation
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