Thalassemia in Sri Lanka

Abstract

The island nation of Sri Lanka with 22 million people (in 2020) has an estimated 2000 patients with severe thalassemia. The majority have β-thalassemia (β-thal) major (β-TM), and Hb E (HBB: c.79G>A)/β-thal accounts for most of the remainder. Carrier rate for α⁺-thalassemia (α⁺-thal) trait is 9.9% and β-thal trait is 2.5%, with very similar rates in the three major ethnic groups (Sinhalese, Tamils and Moors). The distribution of thalassemia type reveals a remarkable variation, even in this small island, mirroring historical distribution of malaria. Even though healthcare is provided free by the state including blood transfusions and chelation, the overall survival of patients of β-TM is still not on a par with that of the Mediterranean countries. A national thalassemia prevention program was set up in 2007, but overall success of the exercise based essentially on dissuasion of marriages is not very promising.

Keywords:

• Hemoglobinopathy
• prevention
• South Asia
• Sri Lanka

Disclosure statement

The authors report no conflicts of interest. The authors alone are responsible for the content and writing of this article.

Additional information

Funding

This Special Issue on Thalassemia in Asia 2021 was partially supported by Project KY202002008 from the Ministry of Science & Technology of the People’s Republic of China through the Asian Thalassemia Training Center (ATTC) at Guangxi Medical University, Nanning, Guangxi, PRC.