Influence of blood transfusion rate on some clinical manifestations in β-thalassaemia major patients

Abstract

Background The commonest form of life-long treatment for individuals with β-thalassaemia major (TM) is blood transfusion; however, regular and multiple transfusion can result in iron overload as well as serious infections. Objectives This study was designed to evaluate clinical manifestations in β-TM patients according to the blood transfusion rate. Methods Forty-two patients of homozygous β-TM in the thalassaemia Division/Children’s Teaching Hospital, Kerbela in the middle part of Iraq, during March–June 2015. All patients (17 males and 25 females) with age range 3–31 years were blood transfusion-dependent and on iron chelation therapy. They were divided into three groups according to the yearly average of blood transfusion received during the last 3 years; those who received less than 16 times/year (G1, n = 16), between 16 and 21 times/year (G2, n = 14) and more than 21 times/year (G3, n = 12). Several biochemical tests were carried out to estimate the serum level of ferritin, total serum bilirubin (TSB), GPT, GOT, alkaline phosphatase (ALP), albumin and total protein. Results The results showed that half of patients were presented either with splenomegaly (28.6%) or splenectomy (21.4%), about one quarter of them (26.2%) were presented with hepatomegaly, more than half of patients (54.8%) were represented with HCV infection and the majority (97.6%) of patients have normal BMI. However, results of biochemical markers revealed that all of patients (100%) have albumin and total protein concentrations respectively, while all of them (100%) and the majority of them (76.2%) have elevated serum level more than the upper limit of normal reference of ferritin and TSB respectively. Additionally, liver enzymes levels (GPT, GOT and ALP) also recorded an elevation in about 31, 42.9 and 21.4% of thalassaemic patients. On the other hand, results demonstrated that increasing in the rate of blood transfusion revealed an association with the reduction in the frequency of patients with normal liver, spleen and negative HCV infection, and the three different groups of thalassaemic patients have significant differences in their age, BMI, rate of transfusion and total protein. However, the rest of biochemical tests (ferritin, TSB, GPT, GOT, ALP and Alb) revealed non-significant differences in their serum levels among the three groups of patients, but the percentage of patients that have elevated ALP were significantly different (0, 35.7 and 33.3%) (P = 0,029) among the G1, G2 and G3 respectively. Conclusion Although blood transfusion is the commonest therapy to improve the life-span of β-thalassaemic patients worldwide, its rate should be reduced as less as possible to avoid the serious complications by searching for another criteria to indicate transfusion rather than haemoglobin concentration such as the antioxidant status of thalassaemic patients.

Keywords

• thalassaemia major, iron overload, liver enzymes, ferritin