Health status of patients with β-thalassemia in the West Bank: a retrospective study

Abstract

BackgroundThe management of β-thalassemia in developing countries is challenging due to few treatments being available other than recurrent transfusions. We assessed the haematological, biochemical, and hormonal findings among patients with β-thalassemia in the West Bank, occupied Palestinian territory. MethodsThis retrospective study was done between Jan 1, 2017, and Dec 31, 2018. Data on haematological, biochemical, and hormonal tests were collected from medical files of patients with β-thalassemia in eight primary health-care clinics, nine emergency departments, and eleven Palestinian Ministry of Health hospitals across the West Bank. Additionally, we collected data on demographics and the use of iron chelation. The study was approved by the research ethics committee at Al-Quds University and the Palestinian Ministry of Health (reference 162/1075/2019). The data had no identifying information and, therefore, informed consent from patients was waived for this study. Findings309 patients with β-thalassemia were included in the study (154 men and 155 women), with an average age of 23·4 (SD 10·4) years. 202 (79%) patients had pretransfusion haemoglobin concentrations lower than 9 g/dL (mean 8·4 [SD 1·4]). Additionally, 185 (73%) had iron overload, with the mean ferritin concentration in serum being 3175·8 μg/L (SD 3378·8). Concentrations of alanine aminotransferase and aspartate aminotransferase were high in 91 (38%) and 145 (61%) patients, respectively, and correlated positively with serum ferritin concentrations (r=0·527 and r=0·254, respectively; both p<0·0001). Kidney function tests (serum creatinine and blood urea nitrogen) did not correlate with serum ferritin (p=0·553 and 0·280, respectively). Deferoxamine was used for iron chelation in 63 (27%), and these patients had significantly higher ferritin concentrations in serum than patients receiving deferasirox (175 [74%], p=0·009). A small proportion of patients had endocrine function data that indicated abnormal total tri-iodothyronine, free thyroxine, and thyroid-stimulating hormone levels in 24 (34%), 29 (37%), and 24 (15%) patients, respectively, in addition to abnormal parathyroid hormone levels in 25 (50%) patients. However, these findings were not sufficient to diagnose or interpret endocrinopathies. InterpretationOur findings showed a lack of adherence to international guidelines in the management of β-thalassemia, which was reflected by the low pretransfusion haemoglobin and high serum ferritin concentrations. This study highlights the importance of establishing patient-tailored comprehensive assessment and follow-up protocols for the management of β-thalassemia with an emphasis on blood transfusion and iron chelation practices. FundingNone.