B-Thalassemia - Call for Restoration of Normal Vitamin E Status

Short Communication

Ann Hematol Oncol. 2022; 9(1): 1390.

β-Thalassemia - Call for Restoration of Normal Vitamin E Status

Wilairat P¹, Auparakkitanon S² and Wilairat P³*

1Department of Chemistry, Faculty of Science, Mahidol University, Bangkok, Thailand

2Department of Pathology, Faculty of Medicine Ramathibodi Hospital, Mahidol University, Bangkok, Thailand

3Department of Biochemistry, Faculty of Science, Mahidol University, Bangkok, Thailand

*Corresponding author: Wilairat P, Department of Biochemistry, Faculty of Science, Mahidol University, Bangkok, 10400 Thailand

Received: February 23, 2022; Accepted: March 12, 2022; Published: March 19, 2022


F2-Isoprostane; Hypercoagulopathy; Oxidative stress; Vitamin E Supplementation; β-Thalassemia

Short Communication

A recent review of β-thalassemia pathophysiology discussed several drugs (old and new) for treating anemia and concomitant iron overload [1], but an obvious consequence of the presence of unmatched a-hemoglobin (Hb) in β-thalassemia red blood cells (β-thal RBCs) is systemic oxidative stress, first noted by reduced plasma vitamin E (vit E) levels and increased sensitivity of β-thal RBCs to H2O2-induced lysis in β-thalassemia subjects [2]. This led to numerous clinical trials in β-thal subjects of vitamin E supplementation (alone or combined with other anti-oxidants, e.g., N-acetyl cysteine), ranging from 300 mg/day for 15 days to 600 mg/day for nine months, which produced improvements in β-thal RBC parameters of oxidant damage but not in Hb levels, dampening this “quick fix” approach [3]. This is not surprising given recent understandings of the complex changes to erythrocyte plasma membrane (including intracellular membranes of erythroid progenitor cells) caused by bound unmatched a-Hb and consequent heme-dependent oxidative damage to both membrane lipids and proteins, affording an explanation for ineffective erythropoiesis (due to apoptosis/autophagy) and premature eryptosis [4]. Although the exact mechanisms by which aged RBCs are removed by the body reticuloendothelial system remain unclear, in the case of β-thal RBCs, oxidative damage to plasma membrane proteins is accepted as the primary etiology.

Nevertheless, a new appraisal for restoration of normal vitamin E status in β-thal patients is warranted. With availability of fluorescentlabelled Annexin V to detect presence of cell surface phosphatidylserine (PS), it was realized that PS on surface of circulating β-thal RBCs is responsible (in part) to the hypercoagulable state, platelet activation, thrombosis and pulmonary hypertension observed in β-thal subjects [1,4]. A limited number of clinical trials of vit E supplement (alone or together with N-acetylcysteine) on small cohorts of β-thal subjects showed improvement in parameters related to oxidative stress and hypercoagulopathy (Table 1). However, it is worth noting that upon cessation of vitamin E supplementation, all measured parameters (including plasma vit E) returned to pre-treatment levels within three months (in studies that carried out these experiments).

Citation: Wilairat P, Auparakkitanon S and Wilairat P. β-Thalassemia - Call for Restoration of Normal Vitamin E Status. Ann Hematol Oncol. 2022; 9(1): 1390.