Immunological Evaluation of Patients with Beta-Thalassemia major

Consolini, Rita; Calleri, Angelica; Legitimo, Annalisa; Massei, Francesco

doi:10.1159/000046526

Cited by 29 publications

(27 citation statements)

References 25 publications

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“…While this outcome has been reported previously (Dwyer et al, 1987;Lombardi et al, 1994), contrasting findings of higher CD8, reduced CD4 and decreased CD4/CD8 ratio were also reported (Umiel et al, 1984;Grady et al, 1985;Ezner et al, 2002). Few studies even reported thalassemia patients with low CD8 percentage (Cunningham-Rundles et al, 2000;Consolini et al, 2001). A study conducted by Cruz et al (2006) reported a negative correlation between CD8+ T-lymphocytes and iron stores in HFE homozygous hemochromatosis male patients; this correlation was not found in our patient population.…”

Section: Discussioncontrasting

confidence: 58%

Investigation of the distribution of lymphocyte subsets and zinc levels in multitransfused β‐thalassemia major patients

Al-Awadhi

AlFadhli

Al-Khaldi

et al. 2010

Int J Lab Hematology

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Homozygous beta-thalassemia is a common genetic disorder in the Arabian Peninsula and an important cause of morbidity in Kuwait. The anemia is so severe that chronic blood transfusions, and the resulting iron overload, cause a shift in immunoregulatory balances and a deficiency in zinc. It was reported that individual immunological profile of CD8+ T-lymphocytes may have a modifying effect on the severity of iron overload in HFE homozygous hemochromatosis patients, with low numbers being negatively correlated with the total amount of body iron stores. This has not been tested in thalassemia major patients. This study was designed to utilize flow cytometric immunophenotyping to characterize effects of regular blood transfusion, and high serum ferritin levels because of irregular use of iron chelation therapy on T lymphocytes (CD2, CD3, CD4 and CD8), B lymphocytes (CD19) and natural killer cells (CD56) and zinc levels in the blood of patients with thalassemia major (n = 49) and healthy normal controls (n = 60) in Kuwait. None of the patients had active infections. T-cell markers' percentage levels were comparable between patients and controls (P > 0.05), while B cell marker (CD19) was significantly higher in patients (P = 0.007). Patients had lower percentage levels of CD56 cells (P = 0.007) and normal serum zinc. All patients had high serum ferritin levels with no significant correlation to CD8+ T lymphocytes (P > 0.05). High iron stores did not have an effect on T lymphocytes' profile, with normal zinc levels perhaps related to non compliance with chelation therapy. The high B cell marker may be indicative of stimulation of antibody producing cells as a result of regular blood transfusions.

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Section: Discussioncontrasting

confidence: 58%

Investigation of the distribution of lymphocyte subsets and zinc levels in multitransfused β‐thalassemia major patients

Al-Awadhi

AlFadhli

Al-Khaldi

et al. 2010

Int J Lab Hematology

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“…In another study, Kwan shows that while serum zinc level decreases and serum zinc binding capacity (ZnBC) increases in nutritional zinc deficiency, in thalassemic patients serum zinc level decreases but ZnBC does not increase simultaneously [22]. Though there are wide variations in different reports, these discrepancies can be attributed to differences in patients' ages, rather than to difference in treatment protocols, including differing transfusion rates or chelation therapies [22][23][24][25][26][27]. Some reports indicate that desferrioxamine increases urinary zinc excretion and may decrease zinc body content [8,9,28].…”

Section: Discussionmentioning

confidence: 99%

ZINC STATUS IN PATIENTS WITH MAJOR Β-Thalassemia

Mehdizadeh

Zamani

Tabatabaee

2008

Pediatric Hematology and Oncology

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To determine the zinc status in patients with major beta-thalassemia and its effect on their growth, the authors studied 64 thalassemic patients in comparison with 64 healthy matched individuals. Demographic and anthropometric data and history of the therapies were collected. Serum zinc level in both groups and ferritin in the thalassemic group were assigned. Interestingly, mean serum zinc level was significantly higher in the thalassemic group. No significant correlation between serum zinc level and short stature, serum ferritin level, desferrioxamine dose, initiating time of blood transfusion, and chelation therapy was found. The study indicates zinc deficiency in thalassemic patients who are on regular blood transfusion is rare and it seems that routine zinc supplementation is not necessary.

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“…One explanation for this low activity can be Zn insufficiency, an essential element required for function of immune cells (29,30). In fact, in used iron chelators in TM, Desferoxamine (DFO), Deferiprone (DFP), and Deferasirox (DFX), all have been shown to lead to Zn deficiency (31).…”

Section: Functional Alternationsmentioning

confidence: 99%

The Role of Immune System in Thalassemia Major: A Narrative Review

Bazi¹,

Shahramian²,

Yaghoobi³

et al. 2017

J Pediatr Rev

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Context: Thalassemia is a genetic disorder of hemoglobin production. Patients with thalassemia major (TM) require regular blood transfusions to keep a compatible hemoglobin level for oxygenating organs. These patients suffer from different complications such as infections, autoimmunity and alloimmunization due to transfusion. Such complications link the immune system to TM pathogenesis. In the present study, we have reviewed the latest data available on interactions of TM pathophysiologic determinants and immune system components. Evidence Acquisition: A comprehensive search was performed on PubMed, Scopous, and Web of Knowledge databases using keywords thalassemia, immune system, autoimmune, alloimmune, adaptive immunity, innate immunity, complications, and immunesenescnce. Results: It seems that persistent antigenic stimulation and oxidative stress from excessive iron are the two main pathophysiologic factors of TM impacting the immune system. Regarding innate immunity, functional activity of neutrophils, and natural killer cells (NKCs) is decreased in TM. On the other hand, higher levels of TNF-α and IL-1β, IL-6, IL-8, and C-reactive protein proinflammatory cytokines have been observed in the serum of patients. TM patients have demonstrated higher ratios of regulatory B lymphocytes (CD19+, CD38+, CD24+), helper T cells, suppressor T cells, and T regulatory (CD4+/CD25+/Foxp3+) lymphocytes. TM patients have shown significant higher levels of IgA immunoglobin respective to normal counterparts that may predispose them to diabetes and coeliac disease. Immune cells, however, rendered lower than optimal activity in TM patients, which may be due to nutritional insufficiencies. Potential relationships have been suggested between immune system and various thalassemia compilations including heart infraction, hypertension, atherosclerosis, diabetes, thyroid dysfunction, and osteoporosis. Conclusions: Immune genetic determinants may be involved in modulating the clinical picture of TM. TM patients generally represents with higher immune cell counts, likely as a result of persistent antigenic challenge from blood transfusions. However, these patients face compromised immune cell functions. The role of immunologic interactions in pathogenesis of TM needs to be further divulged in future studies.

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Immunological Evaluation of Patients with Beta-Thalassemia major

Cited by 29 publications

References 25 publications

Investigation of the distribution of lymphocyte subsets and zinc levels in multitransfused β‐thalassemia major patients

Investigation of the distribution of lymphocyte subsets and zinc levels in multitransfused β‐thalassemia major patients

ZINC STATUS IN PATIENTS WITH MAJOR Β-Thalassemia

The Role of Immune System in Thalassemia Major: A Narrative Review

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