Evaluation of immune system in patients with transfusion‐dependent beta‐thalassemia in Rasoul‐e‐Akram Hospital in 2021: A descriptive cross‐sectional study

Abstract

Background and aims Thalassemia syndromes are the most common hemoglobinopathy globally related to blood transfusion and iron overload in the body. Splenectomy, excessive iron overload, and repeated exposure to antigens in blood transfusions can cause severe damage to the patient's immune system making the patient prone to frequent infection. This study evaluates the immune system status and infection rate in beta‐thalassemia major patients receiving iron chelators. Methods This descriptive cross‐sectional study was performed in Rasoul‐e‐Akram Hospital on patients with a beta‐thalassemia major who had iron overload due to frequent blood transfusions. The percentage of lymphocyte markers was determined by flow cytometry. Serum levels of immunoglobin were measured by nephelometric assay. Also, Nitro blue tetrazolium and dihydrorhodamine assays were used to evaluate the phagocytic function. Results Of the 106 patients participating in this study, 59 (55.7%) and 47 (44.3%) are male and female, respectively. The mean age ± SD of participants was 24.7 ± 12.1 years with 4 to 55 years. There was no significant correlation between sex, the C3 and C4 complements, the lymphocyte markers, and the immunoglobulin levels. Furthermore, all of these variables increased significantly over 30 ( p < 0.05). Moreover, there was a strong positive correlation between splenectomy and IgG immunoglobulin ( p < 0.001) and CD16 ( p = 0.005) lymphocyte marker. Conclusion Iron chelator agents effectively improve patients' immune system with thalassemia major. The increase in IgG and IgM immunoglobulins levels is due to frequent blood transfusions, which stimulate the immune system.

Hearing loss in beta‐thalassaemia: An Italian multicentre case–control study

“…The literature prevalence rate, also in very recent studies, 6,7 was substantially confirmed in our study: merging hypoacusis and sensorineural notch, hearing deficits were found in 38% of patients, with hypoacusis (PTA >25 dB) in about one out of four (26.6%) and sensorineural hypoacusis in about one out of five patients (21%).…”

Complications in patients with transfusion dependent thalassemia: A descriptive cross‐sectional study

“…The most severe form of beta‐thalassemia, known as Cooley's anemia, is characterized by a lack of beta protein in hemoglobin and requires frequent blood transfusions for survival 3–5 . Complications associated with beta‐thalassemia include iron overload, splenomegaly, growth retardation, immune system disorders, heart and renal failure, and liver disease 6,7 . Iron overload is a major concern for patients with TDT, as it can lead to severe complications such as endocrinopathy, cardiomyopathy, diabetes, auditory abnormalities, osteoporosis, and growth failure 6,8 .…”

“…3 , 4 , 5 Complications associated with beta‐thalassemia include iron overload, splenomegaly, growth retardation, immune system disorders, heart and renal failure, and liver disease. 6 , 7 Iron overload is a major concern for patients with TDT, as it can lead to severe complications such as endocrinopathy, cardiomyopathy, diabetes, auditory abnormalities, osteoporosis, and growth failure. 6 , 8 This occurs due to increased intestinal absorption of iron due to erythropoiesis, leading to its accumulation in various organs.…”

The relationship between immunologic system and ferritin levels in patients with transfusion dependent thalassemia: a retrospective single-center study

“…In the study conducted by Ehsanipour et al 12 in 106 patients aged between 4 and 50 years, many parameters, including complement levels, lymphocyte markers, immunoglobulin levels, use of chelator drugs, ferritin, and age, were examined. Similar to our study, lymphocyte markers were also examined in patients, and similar to our study, no significant correlation was found between splenectomy, ferritin levels, and lymphocyte markers.…”