Thalassemia Reports

Background/Objectives: Thalassemias, a hereditary condition commonly linked to chronic anemia, require regular blood transfusions and repeated blood draws for assessments of hemoglobin (Hb) content, which can be uncomfortable. A promising substitute for laboratory hemoglobin testing is non-invasive spectrophotometric hemoglobin (SpHb) monitoring; however, its applicability particularly among blood transfusion-dependent thalassaemic patients needs to be investigated. This study’s primary goal was to investigate the relationships and agreements between SpHb, g/dL, and an automated hematology analyzer (Hb, g/dL) in this particular patient population. The secondary goal was to track how blood transfusions affect SpHb, g/dL, laboratory Hb, and pleth variability index (PVI, %). Methods: In this study, sixty patients were included. A Masimo Radical-7 pulse CO-oximeter was used to measure the SpHb, and a Sysmex XN-1000 hematological analyzer measured the laboratory Hb. Results: The results revealed a significant correlation between SpHb and laboratory Hb (n = 108, r = 0.587, p < 0.001) but also demonstrated that SpHb consistently overestimated laboratory Hb levels, with a mean bias of −1.18 g/dL (95% CI: −1.4344 to −0.9267). The Bland–Altman analysis showed a good degree of reliability between this bias (SpHb–Hb) and laboratory Hb (g/dL), with an Intra Class Correlation (ICC) of 0.613 but with a wide 95% CI ranging from 0.557 to 0.736 (t = 3.817, p < 0.001). The 95% limits of agreement ranged from −3.7893 to +1.4228 g/dL. Conclusions: This significant bias restricted the application of SpHb as a trustworthy method for assessing hemoglobin levels in patients with blood transfusion-dependent thalassemia. Nonetheless, the capability to monitor SpHb and PVI variations during blood transfusions offered a real-time assessment of the impact of transfusions on patients’ hemoglobin levels and volume status.

Background: It has been shown that human leucocyte antigen (HLA) alleles are related to certain diseases. Some alleles were associated with alloimmunization in individuals with thalassemia. In this study, we studied the distribution of HLA alleles among beta thalassemia (BT) patients compared to healthy controls. Material and Methods: The HLA results of 100 patients with BT and 100 healthy controls were obtained for the study. The HLA-A, -B and -DRB1 tissue typing were performed at the laboratory. The low-resolution sequence-specific primer (SSP)–polymerase chain reaction (PCR-SSP) (Olerup HLA-A,B,DR typing kit, USA) and sequence-specific oligonucleotide (SSO)–PCR (LABType HLA-A,B,DR kit, ABD) methods were performed using the Luminex genotyping kits. All related data were retrospectively analyzed. Results: One in five patients (21%) underwent hematopoietic stem cell transplantation (HSCT). Patients with HSCT had significantly lower frequency of HLA-B *14, HLA-DRB1 *11 and HLA-DRB1 *16 alleles and had a higher frequency of HLA-A *66, HLA-B *41, HLA-B *55, HLA-DRB1 *3 alleles compared to patients without HSCT (p < 0.05). The HLA-A *3, HLA-B *41 and HLA-B *55 alleles were more commonly seen in HSCT patients compared to the healthy group (p = 0.04). Female patients showed a higher frequency of HLA-B *58 and HLA-DRB1 *4 alleles (p = 0.04). Conclusions: This study demonstrated that HLA-B *41 and -B *55 alleles were closely related to HSCT among BT patients. It might be considered that the variance in certain HLA-B alleles in BT patients might cause difficulty in finding a matched donor in this limited population.

The risk of anemia and iron overload is a global concern in beta (β)-thalassemia. The β-thalassemia primary treatment includes blood transfusion and iron chelation therapy; however, both are associated with risks such as anemia, iron depletion, overload, and oxidative stress if not adequately monitored. Therefore, this study investigates the effects of curcumin on anemia, iron overload, and oxidative stress in β-thalassemia. In this meta-analysis, search terms including “curcumin,” “Curcuma longa,” “curcuminoids,” “turmeric,” and “thalassemia” were used in Scopus and PubMed to identify studies published from inception to 15 February 2025. The quantitative analysis was performed using a meta-analysis web tool, and the effect estimates were reported as the mean difference (MD) or standardized mean difference (SMD), along with 95% confidence intervals (CI). Our analysis showed no significant effect on hemoglobin (p = 0.1788) and red blood cell count (p = 0.9534). In contrast, there was a significant decrease in serum ferritin [SMD = −0.24 (−0.46, −0.02), p = 0.0335], non–transferrin bound iron (NTBI), [SMD = −0.59 (−0.98, −0.19), p = 0.0039] and serum iron, [SMD = −0.30 (−0.60, −0.01), p = 0.0425]. Furthermore, there was a reduction in reactive oxygen species; [SMD = −0.83 (−1.23, −0.44), p < 0.0001] and malonaldehydes, [MD = −343.85 nmol/g Hb (−465.94, −221.76), p < 0.0001]. A dose of 500 mg of curcumin was found to be more effective in reducing the NTBI. The findings suggest that curcumin may help reduce iron overload and oxidative stress in β-thalassemia; however, its effect on improving anemia appears to be limited. Given the small sample size of the included studies, we recommend that future research involve larger cohorts and employ rigorous methodologies to evaluate the therapeutic potential of curcumin in β-thalassemia thoroughly. Additionally, we recommend using curcumin-enhancing strategies to improve its bioavailability and administer an optimal yet effective dose.