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World Journal of Anemia
Abstract
 
 

ABSTRACT

Thalassemia, also known as Mediterranean anemia, can be considered as the most common monogenetic disease prevailing all across the world. This disorder involves production of abnormal amounts of hemoglobin in the body, which poses a significant burden on the health and economic status of the patients as well as their families. Generally, patients with the thalassemia trait have a normal life expectancy, but individuals with beta thalassemia major mostly die from cardiac complications due to iron overload by the time they reach 30 years of age. Each year, nearly 70,000 babies are born with thalassemia worldwide. Conventional treatment procedures available (e.g., lifelong red blood cell transfusion, iron chelation therapy, and splenectomy) have levied high expenses on the health-care systems.
Thalassemia during pregnancy could be associated with significant complications to the mother as well as her fetus. Therefore, universal antenatal screening for thalassemia carriers should be implemented in populations having a high prevalence of this condition. In order to improve survival among children born with thalassemia, there is a requirement for combined treatment and prevention program during pregnancy. Preconception genetic counseling is strongly advised for all patients with thalassemia. Among the high-risk parents, the most important method for diagnosis of thalassemia is invasive prenatal diagnosis. Following a standard management plan and close monitoring of the maternal and fetal condition during pregnancy helps in considerably reducing the mortality and morbidity associated with this condition.
Novel treatment approaches are recently being developed to correct the resulting α/β globin chain imbalance, in an effort to move beyond the palliative management of this disease and tackle the exact genetic defect involved in its pathogenesis. Three methods for medical treatment of thalassemia have been envisioned: fetal globin gene renaissance by pharmacological compounds being injected into patients, allogeneic hematopoietic stem cell transplantation, and gene therapy. These medical strategies can be considered as the best options prevailing now and are currently under research and clinical studies.

Keywords: α-thalassemia, β-thalassemia, Allogeneic hematopoietic stem cell transplantation, Gene therapy, Iron chelation, Pregnancy management, Splenectomy, Thalassemia

How to cite this article: Saxena R, Banerjee T, Aniyery RB. Thalassemia and its Management during Pregnancy. World J Anemia 2017;1(1):5-17.

Source of support: Nil

Conflict of interest: None

 
 
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