Benefits and Complications of Regular Blood Transfusion in Patients with Beta-Thalassaemia major

Abstract

Early and regular blood transfusion therapy in patients with homozygous β-thalassaemia decreases the complications of severe anaemia and prolongs survival. In the long term, however, the beneficial effects of transfusions are limited by the organ damage resulting from iron overload, a consequence of the body’s limited capacity to excrete iron, and by the complications of infection with blood-borne agents. Transfusion regimens for β-thalassaemia have changed substantially during the past four decades. In current protocols, pre-transfusion haemoglobin concentration should not exceed 95 g/l. This allows adequate control of anaemia, with a relatively low rate of iron accumulation. Although iron chelation therapy has successfully improved survival free from cardiac disease, thalassaemic patients continuously present new clinical challenges. In fact, the vast majority of them suffer from post-transfusion chronic hepatitis C, which is expected to significantly contribute to morbidity in the forthcoming years. Furthermore, recent studies demonstrated that thalassaemics are at high risk of acquiring several blood-borne viruses. The potential role of these multiple infections in inducing clinical disease is still uncertain, and needs to be thoroughly clarified in future surveys.

Prevalence of hepatitis B and hepatitis C virus infection in repeatedly transfused thalassemics in a tertiary care hospital in eastern India

“…Apart from iron toxicity, hypersplenism, venous thrombosis and osteoporosis, these patients are confronted by new clinical challenges, particularly in the form of transfusion transmitted diseases, especially hepatitis B virus (HBV), hepatitis C virus (HCV) and human immunodeficiency virus (HIV) infections. 4,5 Post-trans fusional viral hepatitis has significantly contributed to morbidity in thalassemia with increased risk for development of hepatocellular carcinoma. 4,6 Hepatitis C virus is a hepatotrophic, single strand ribonucleic acid (RNA) virus of family Flaviviridae and genus hepacivirus.…”

“…4,5 Post-trans fusional viral hepatitis has significantly contributed to morbidity in thalassemia with increased risk for development of hepatocellular carcinoma. 4,6 Hepatitis C virus is a hepatotrophic, single strand ribonucleic acid (RNA) virus of family Flaviviridae and genus hepacivirus. An estimated 180 million people worldwide are chronically infected with hepatitis C with the global prevalence being around 2% and 3 to 4 million persons getting newly infected each year.…”

Red Blood Cell Antibodies in Thalassemia Patients in Northern India: Risk Factors and Literature Review

“…Beta thalassemia major usually manifests within the first year of life in 95 % of the patients and these patients are dependent on life long transfusion therapy for their survival. Transfusion therapy has been a key intervention in decreasing the thalassemia disease associated morbidity and mortality and it still remains a mainstay of treatment for majority of thalassemia patients despite the successful use of hematopoietic stem cell transplantation to cure the disease [3]. However, with multiple intermittent or chronic transfusions these patients are exposed repeatedly to risks associated with transfusion therapy i.e., infectious disease transmission, volume overload, haemolytic transfusion reactions, iron overload, increased risk of allo and autoimmunization and clinically significant delayed haemolytic transfusion reactions (DHTRs) [3,4].…”

Age at infection affects the long-term outcome of transfusion-associated chronic hepatitis C

“…In Italy, more than 10% of the patients receiving blood transfusion during the 1980s developed clinically evident posttransfusion hepatitis, 2,3 and the prevalence of anti-HCV reactivity among transfusion-dependent patients starting their transfusion regimens in the same period approached 90%, with 70% of these patients developing chronic HCV infection. 4,5 The current risk of acquiring HCV infection through blood transfusion is negligible in comparison with other routes of viral transmission 4,6 and can be expected to decrease further following the adoption of nucleic acid testing technology for blood screening. However, specialists in various fields of medicine, including hematologists and hepatologists, are now facing the clinical effects of the past epidemic of transfusion-associated HCV.…”