Abstract

Inherited thalassaemia disease is commonly found in many countries of the world. Care of the disease requires comprehensive management strategies comprising of clinical management of both transfusion dependant thalassaemia (TDT) and non-transfusion dependant thalassaemia (NTDT). It also includes preventive measures such as screening programmes and genetic counseling in order to contain the genetic transmission. At the moment, the only cure is through haematopoeitic stem cell transplant (HSCT). This report illustrates thalassaemia disease prevalence in the Terengganu state and the evolution of care since National Thalassaemia Programme was launched in 2009.

Highlights

  • Thalassaemia disease is well known to many parts of the world especially in the region of “thalassaemia belt” which stretches from Mediterranean countries going through subSaharan Africa and Middle East to South and South East Asia including Malaysia [1]

  • The clinical presentation range from non-transfusion/infrequent transfusion disease to a total transfusion dependant thalassaemia (TDT)

  • Since Kuala Terengganu is the major district in Terengganu, it has the largest number of thalassaemia patients, 225 (65.4%)

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Summary

Introduction

Thalassaemia disease is well known to many parts of the world especially in the region of “thalassaemia belt” which stretches from Mediterranean countries going through subSaharan Africa and Middle East to South and South East Asia including Malaysia [1]. The clinical presentation range from non-transfusion/infrequent transfusion disease to a total transfusion dependant thalassaemia (TDT). The latter type confers a much predicted morbidities & mortality from the iron loading as a result of regular blood transfusion. In Malaysia, HbE beta thalassaemia and beta thalassaemia major constitute the two major types of thalassaemia disease, comprising of 34.4% and. Among the states in Malaysia, thalassaemia patients in Terengganu constitutes close to 5% of the total thalassaemia patients in the country (Figure 1)[3].

Penang Perlis
Disease Pattern
Iron Burden
Preventive Measures
Findings
Current Management Strategy
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