Issues with Enhanced Longevity Among Patients with Beta-Thalassemia
November 2021
The annual incidence of beta-thalassemia is approximately 1 in 100,000 with significant regional variations globally. It has decreased over time especially with the introduction of national-wide screening programs. In Taiwan, the prevalence decreased from 5.6 in 1994 to 1.2 per 100,000 births in 2002.
The diagnosis of beta-thalassemia may entail life-long regular transfusion for survival and the terminology, transfusion-dependent beta-thalassemia, is conventionally used to describe such form.
A group of researchers sought to understand the relationship between transfusion burden and the development of issues and health care resource utilization (HCRU) among patients with beta-thalassemia. They found that each red blood cell transfusion (RBCT) unit increase was significantly and positively correlated with HCRU related to thalassemia and an enhanced risk of liver, endocrine, cardiac, and renal complications.
Enhancements in preventative measures have substantially increased life expectancy of patients with beta-thalassemia. Yet, longer survival led to the emergence of its own set of unrecognized issues. Underlying pathophysiology of the disease has not been eliminated, and transfusion, although effective, may be a major source of morbidity. As such, standards must be established and maintained to ensure a safe approach to the utilization of transfusions in the management of beta-thalassemia.