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2000
Volume 10, Issue 1
  • ISSN: 1871-5257
  • E-ISSN: 1875-6182

Abstract

Hemoglobin disorders, including thalassemia and sickle cell disease, are prevalent in populations that evolved in moderate and humid climates where malaria was endemic. Thalassemia is an inherited, autosomal recessive disease that involves a reduced rate of synthesis of a globin chain that is essential for hemoglobin synthesis. This reduced synthesis can cause abnormal hemoglobin molecules to form, leading to anemia in humans. Thalassemia and sickle cell disease affect all ethnic populations. Extensive literature on these disorders suggests that α-thalassemia and sickle cell trait that are known to protect against malaria. β-thalassemia is associated with people of Mediterranean origin and the Indian subcontinent, particularly Northern India and Pakistan. Sickle cell disease is prevalent on the African continent and, to a greater extent, in tribal areas of central and southern of India. In the last 3 decades, tremendous progress has been made in understanding of hemoglobin disorders, including the nature and types of hemoglobin diseases, animal models that can be used to advance our understanding of these diseases, possible early diagnosis of β-thalassemia that can be treated with blood transfusions, and finally the risks of repeated blood transfusions for patients with β-thalassaemia and sickle cell disease. The purpose of this special topic was to assess the current understanding of thalassemias and sickle cell disease and to review advancements in diagnosing and treating them, including a review of risks associated with blood transfusion, including iron chelating agents - as emerging treatments for these diseases. It is my hope that this issue provides timely resource for all those who are interested in thalassaemias and sickle cell disease. I am most grateful to all the contributors and outstanding reviewers for their efforts in making this special issue a reality. Sincere thanks also to the Editor, Dr. Debabrata Mukherjee, the Editorial manager, Mr. Rao G. Hussain and the production team of Cardiovascular & Hematological Agents in Medicinal Chemistry for their professional support and the opportunity to assemble this special issue.

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/content/journals/chamc/10.2174/187152512799201154
2012-03-01
2025-09-04
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  • Article Type:
    Research Article
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