Cardiovascular evaluation and management of iron overload cardiomyopathy in sickle cell disease

Ginwalla, Mahazarin; Almasoud, Abdullah; Tofovic, David; Alin, Tara; Al‐Kindi, Sadeer; Oliveira, Guilherme H.; Rajagopalan, Sanjay; Schilz, Robert; Little, Jane A.

doi:10.1002/ajh.24924

Cited by 12 publications

(7 citation statements)

References 6 publications

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“…The ability to mitigate the risk of excessive iron deposition in SCD is clinically important. Excessive iron stores have been associated with liver fibrosis 6,[16][17][18] cardiomyopathy [19][20][21] and mortality. 6,18,22 If excessive iron stores are not prevented, the risk benefit ratio of regular transfusion therapy is attenuated because the benefit of transfusions to prevent SCD complications must be balanced with the long-term risks of the therapy.…”

Section: Discussionmentioning

confidence: 99%

Automated exchange compared to manual and simple blood transfusion attenuates rise in ferritin level after 1 year of regular blood transfusion therapy in chronically transfused children with sickle cell disease

Kelly

Rodeghier²,

DeBaun

2020

Transfusion

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Background: Optimal strategies for regular blood transfusion therapy are not well defined in sickle cell disease (SCD). This analysis tested the hypothesis that in the first of year of regular transfusions, when chelation therapy use is minimal, automated exchange transfusion would be the superior method for attenuating the rise in ferritin levels compared to simple and manual exchange transfusions. Study design and methods: The Silent Cerebral Infarct Multi-Center Clinical Trial randomly allocated children with SCD and silent cerebral infarcts to receive standard care or regular transfusions with a target pre-transfusion HbS concentration ≤ 30% and minimum hemoglobin level > 9.0 g/dL. Participants with at least nine transfusions and sufficient ferritin testing in the first year of the trial were included in a planned secondary analysis. Ferritin levels by the end of the first study year were compared between participants receiving automatic exchange transfusion, manual exchange transfusion, and simple transfusion. Results: A total of 83 participants were analyzed. During the first year of the study, 75.9% of the participants had >80% of transfusions via one transfusion method. At baseline no significant differences in ferritin levels were observed in the three transfusion groups (p = 0.1). After 1 year of transfusions the median (interquartile range) ferritin levels in the simple transfusion (n = 40), manual exchange transfusion (n = 34) and automatic exchange transfusion (n = 9) groups were 1800 ng/mL (1426-2204 ng/mL), 1530 ng/mL (1205-1805 ng/mL), and 355 ng/mL (179-579 ng/mL), respectively (p < 0.001). Conclusion: Automated exchange transfusion, when compared to other transfusion methods, is the optimal transfusion strategy for attenuating increase in ferritin levels in children with SCD.

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Section: Discussionmentioning

confidence: 99%

Automated exchange compared to manual and simple blood transfusion attenuates rise in ferritin level after 1 year of regular blood transfusion therapy in chronically transfused children with sickle cell disease

Kelly

Rodeghier²,

DeBaun

2020

Transfusion

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“…Two out of seven had heart failure with reduced ejection fraction (HFrEF), while five had heart failure with preserved ejection fraction (HFpEF). Iron chelation therapy helped patients with HFrEF in partially recovering their ejection fraction [14]. Similarly, the other study that included 201 SCD patients revealed only five (2.5%) patients showed myocardial iron overload [15].…”

Section: Myocardial Iron Overload Uncommon In Scdmentioning

confidence: 90%

“…However, many studies were able to prove that iron overload is not a common phenomenon, as seen on the cardiac resonance imaging (CMR) using T2* imaging in patients with SCD [6,13]. Myocardial iron deposition may only occur in 2%-5% of SCD patients with chronic blood transfusions, which may lead to systolic dysfunction, heart failure, arrhythmias, and sudden death in patients when present [14,15]. The first study showed that out of 200 homozygous SCD patients that they studied, only seven had myocardial iron deposits.…”

Section: Myocardial Iron Overload Uncommon In Scdmentioning

confidence: 99%

Cardiomyopathy in Sickle Cell Disease

Kaur¹,

Aurif²,

Kittaneh³

et al. 2020

Cureus

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Sickle cell disease (SCD) is an inherited disorder that occurs due to point mutation in the betaglobin chain resulting in the production of hemoglobin S that tends to become rigid and sickleshaped under low oxygen concentration. These sickle-shaped red blood cells (RBCs) obstruct the blood vessels leading to reduced blood flow to the organs, causing ischemia and tissue fibrosis. These sickle RBCs being abnormal in shape are frequently sequestered by the spleen, creating a state of chronic anemia in the body. This chronic anemia leads to a high cardiac output state causing cardiac remodeling. To tackle chronic anemia, patients are frequently treated with blood transfusions that makes them more prone to the risk of iron overload (from newly transfused RBCs and iron release from the RBCs that just got sequestered as well as from volume overload) and volume overload causing left ventricular (LV) dilation. The abovementioned mechanism of cardiac hypertrophy, along with LV dilation together, makes SCDrelated cardiomyopathy unique cardiomyopathy with features of restrictive cardiomyopathy with LV dilation. It is interesting to note here that even though there is a presence of LV dilatation, Systolic dysfunction is very uncommon in SCD-related cardiomyopathy.

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“…Excess iron accumulates in the parenchymal tissues of different organs and causes degenerative lesions due to its toxicity. 31,34,35 Less than 10% of SCD-transfused received more than twenty RBCs units during follow-up. All these patients had a very high ferritin level (> 1000ng/ml).…”

Section: Risk Factors Occurred Post-transfusion Complicationsmentioning

confidence: 99%

Transfusion Practice, Post-Transfusion Complications and Risk Factors in Sickle Cell Disease in Senegal, West Africa.

Seck

Senghor²,

Loum³

et al. 2022

Mediterr J Hematol Infect Dis

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Context and Objectives: Blood transfusions (BT) remain a mainstay of therapy for patients with sickle cell disease (SCD), but pose significant clinical challenges. We aim to assess infectious markers, red cell alloimmunization and iron overload secondary to BT in SCD patients. Materials and Methods: This is a case-control study included 253 SCD (153 SCD-transfused and 100 SCD non-transfused). We evaluated the transfusion practice (modalities, indications), post-transfusion complications (infections, alloimmunization, iron overload) and risk factors of these complications (socio-demographic, clinical, biological). Results: Median age was 28.5 years (5 - 59). Sex ratio was 0.86. Homozygous SCD was more common (95.3%). Simple BT was performed in 92.8% and transfusion exchange in 18.9%. Transfusion indications were dominated by acute anemia (57.06%) and vaso-occlusive crisis (VOCs) (14%). Red blood cell concentrates (RBC) were administered to 93.46%. Median number of RBC received per patient was 10 (2 - 48). The prevalence of VHC in SCD-transfused was 1.33% and 2% for VHB. Anti-HIV antibodies were not found. Red cell alloimmunization frequency was 16%. The most common alloantibodies were anti-rhesus (34.19%) and anti-Kell (23.67%). Iron overload was detected in 7.84%. The number of RBC transfused was the only risk factor for alloimmunization (p = 0.03) and iron overload (p = 0.023). BT frequency was not related to infectious transmission. Conclusion: Despite advances in blood safety, BT therapy is still a risk for SCD polytransfused patients. Although infectious transmission has rare, the risk of alloimmunization and iron overload is high in these patients.

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Cardiovascular evaluation and management of iron overload cardiomyopathy in sickle cell disease

Cited by 12 publications

References 6 publications

Automated exchange compared to manual and simple blood transfusion attenuates rise in ferritin level after 1 year of regular blood transfusion therapy in chronically transfused children with sickle cell disease

Automated exchange compared to manual and simple blood transfusion attenuates rise in ferritin level after 1 year of regular blood transfusion therapy in chronically transfused children with sickle cell disease

Cardiomyopathy in Sickle Cell Disease

Transfusion Practice, Post-Transfusion Complications and Risk Factors in Sickle Cell Disease in Senegal, West Africa.

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