Erythropoiesis is not equally suppressed in transfused males and females with   thalassemia major: are there clinical implications?

Hapgood, Greg; Walsh, Timothy; Cukierman, Ronit; Paul, Eldho; Cheng, Ken Sheng; Bowden, Donald Keith

doi:10.3324/haematol.2014.118216

Cited by 6 publications

(10 citation statements)

References 11 publications

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“…In fact, compared with patients without EMH, they needed to start their first transfusion and undergo regular transfusions and chelation therapy significantly later and had significantly lower ferritin levels and cardiac and liver iron accumulation. Our data are consistent with those of a recent study demonstrating important differences not only in pretransfusion indices of erythropoiesis such as erythropoietin levels, reticulocyte, and nucleated red blood cell counts, but also in the frequency of paraspinal EMH comparing men with women [37]. Using a segmental approach in the heart, we found a significantly higher prevalence of patients with no MIO or heterogeneous MIO and no significant global heart iron among the EMH positive patients.…”

Section: Discussionsupporting

confidence: 92%

Extramedullary hematopoiesis is associated with lower cardiac iron loading in chronically transfused thalassemia patients

et al. 2015

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The aim of this study was to evaluate, in a large cohort of chronically transfused patients, whether the presence of extramedullary hematopoiesis (EMH) accounts for the typical patterns of cardiac iron distribution and/or cardiac function parameters. We retrospectively selected 1,266 thalassemia major patients who had undergone regular transfusions (611 men and 655 women; mean age: 31.3 6 8.9 years, range: 4.2-66.6 years) and were consecutively enrolled within the Myocardial Iron Overload in Thalassemia network. The presence of EMH was evaluated based on steady-state free precession sequences; cardiac and liver iron overloads were quantified using a multiecho T2* approach; cardiac function parameters and pulmonary diameter were quantified using the steady-state free precession sequences; and myocardial fibrosis was evaluated using the late gadolinium enhancement technique. EMH was detected in 167 (13.2%) patients. The EMH1 patients had significantly lower cardiac iron overload than that of the EMH2 patients (P 5 0.003). The patterns of cardiac iron distribution were significantly different in the EMH1 and EMH2 patients (P < 0.0001), with a higher prevalence of patients with no myocardial iron overload and heterogeneous myocardial iron overload and no significant global heart iron in the EMH1 group EMH1 patients had a significantly higher left ventricle mass index (P 5 0.001) and a significantly higher pulmonary artery diameter (P 5 0.002). In conclusion, in regularly transfused thalassemia patients, EMH was common and was associated with a thalassemia intermedia-like pattern of cardiac iron deposition despite regular transfusion therapy.

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Section: Discussionsupporting

confidence: 92%

Extramedullary hematopoiesis is associated with lower cardiac iron loading in chronically transfused thalassemia patients

et al. 2015

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“…A higher pre-transfusion hemoglobin level of 12 to 12 g/dl is appropriate for patients with cardiac complications or other medical conditions [9]; therefore, the presence of complications associated with TDT is a positive predictor of increased blood transfusion utilization. Similar to other studies [51,54,58], splenectomy and female sex were associated with an expected decrease in the number of blood transfusion units compared to male sex and no splenectomy. As mentioned earlier, the normal hemoglobin level for women is less than that of men; therefore, female patients required less blood volume to achieve normal hemoglobin levels than male patients.…”

Section: Discussionsupporting

confidence: 88%

“…This variation was caused by the different patient age groups in the two studies. The number of blood transfusion units was higher in men than in women [51]. This finding could be explained by the fact that the normal hemoglobin level of males is higher than that of females, which suggests that males require a higher blood volume to achieve their normal hemoglobin level.…”

Section: Discussionmentioning

confidence: 90%

Healthcare resource utilization and direct costs of transfusion-dependent thalassemia patients in Dubai, United Arab Emirates: a retrospective cost-of-illness study

Alshamsi

Hamidi

Narcı

2022

BMC Health Serv Res

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Background Patients with transfusion-dependent thalassemia (TDT) require lifelong blood transfusions and iron chelation therapy. Thus, patients afflicted with TDT often have to undergo blood transfusion and iron chelation therapy, which causes a major economic burden on them. However, this topic has not been reported in Dubai, United Arab Emirates (UAE). Hence, this study aimed to evaluate healthcare resource utilization and associated direct costs related to patients with TDT in Dubai, UAE. Methods For this study, a retrospective prevalence-based cost-of-illness analysis based on the UAE healthcare system and patient perspectives was conducted among patients with TDT treated at the Dubai Thalassemia Center in 2019. Information regarding healthcare resource utilization and direct medical costs was collected from the billing system connected to the electronic medical record system. Patients and their families were interviewed for direct non-medical cost estimations. Results A total of 255 patients with TDT were included in the study. The mean annual direct medical cost was estimated at AED 131,156 (USD 35,713) (95% CI: 124,735 – 137,578). The main driver of the medical cost for the participants as iron chelation therapy AED 78,372 (95% CI: 72,671 – 84,074) (59.8%), followed by blood transfusions, which accounted for AED 34,223 (95% CI: 32,854 – 35,593) 26.1% of the total direct medical costs. The mean annual direct non-medical costs was AED 2,223 (USD 605) (95% CI: 1,946 – 2,500). Age (p < 0.001), severe serum ferritin levels (p = 0.016), the presence of complications (p < 0.001), and the type of iron chelation therapy (p < 0.001) were significant predictors of higher direct medical costs incurred by the participants. Conclusion Transfusion-dependent thalassemia poses a substantial economic burden on the healthcare system, patients, and their families. Our results show that the highest medical cost proportion was due to iron chelation therapy. In this regard, efforts must be made to improve the patients’ acceptance and satisfaction with their iron chelation therapy to increase their compliance and improve the effectiveness of treatment, which could play an essential role in controlling the economic burden of this disease. Moreover, greater support is essential for families that suffer catastrophic out-of-pocket expenses.

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“…Erythropoietin levels (EPO) were higher in men (72 mIU/mL) compared to women (52 mIU/mL; p -value = 0.006). The incidence of splenectomy was also higher in men (61% versus 40% in women; p -value = 0.031) 15 . (B)…”

Section: What Is the Purpose And Recommended Regimen Of Transfusions mentioning

confidence: 99%

“…The development of one or more specific anti-red blood cell antibodies (alloimmunization) is an important complication of regular transfusion therapy 13, 14, 15. (B)…”

Section: What Is the Purpose And Recommended Regimen Of Transfusions mentioning

confidence: 99%

Guidelines on Beta-thalassemia major – regular blood transfusion therapy: Associação Brasileira de Hematologia, Hemoterapia e Terapia Celular: project guidelines: Associação Médica Brasileira – 2016

Langhi

Ubiali²,

Marques

et al. 2016

Revista Brasileira de Hematologia e Hemoterapia

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Erythropoiesis is not equally suppressed in transfused males and females with thalassemia major: are there clinical implications?

Cited by 6 publications

References 11 publications

Extramedullary hematopoiesis is associated with lower cardiac iron loading in chronically transfused thalassemia patients

Extramedullary hematopoiesis is associated with lower cardiac iron loading in chronically transfused thalassemia patients

Healthcare resource utilization and direct costs of transfusion-dependent thalassemia patients in Dubai, United Arab Emirates: a retrospective cost-of-illness study

Guidelines on Beta-thalassemia major – regular blood transfusion therapy: Associação Brasileira de Hematologia, Hemoterapia e Terapia Celular: project guidelines: Associação Médica Brasileira – 2016

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