Effectiveness of red blood cell exchange, partial manual exchange, and simple transfusion concurrently with iron chelation therapy in reducing iron overload in chronically transfused sickle cell anemia patients

Fasano, Ross M.; Leong, Traci; Kaushal, Megha; Sagiv, Eyal; Luban, Naomi L.C.; Meier, Emily Riehm

doi:10.1111/trf.13558

Cited by 45 publications

(62 citation statements)

References 31 publications

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“…Our finding of severe iron overload with LIC elevation in patients with SCA managed using erythrocytapheresis contrasts with previous reports [10, 28, 29]. Several factors may explain this discrepancy.…”

Section: Discussioncontrasting

confidence: 99%

Cardiac iron overload in chronically transfused patients with thalassemia, sickle cell anemia, or myelodysplastic syndrome

et al. 2017

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The risk and clinical significance of cardiac iron overload due to chronic transfusion varies with the underlying disease. Cardiac iron overload shortens the life expectancy of patients with thalassemia, whereas its effect is unclear in those with myelodysplastic syndromes (MDS). In patients with sickle cell anemia (SCA), iron does not seem to deposit quickly in the heart. Our primary objective was to assess through a multicentric study the prevalence of cardiac iron overload, defined as a cardiovascular magnetic resonance T2*<20 ms, in patients with thalassemia, SCA, or MDS. Patient inclusion criteria were an accurate record of erythrocyte concentrates (ECs) received, a transfusion history >8 ECs in the past year, and age older than 6 years. We included from 9 centers 20 patients with thalassemia, 41 with SCA, and 25 with MDS in 2012-2014. Erythrocytapharesis did not consistently prevent iron overload in patients with SCA. Cardiac iron overload was found in 3 (15%) patients with thalassemia, none with SCA, and 4 (16%) with MDS. The liver iron content (LIC) ranged from 10.4 to 15.2 mg/g dry weight, with no significant differences across groups (P = 0.29). Abnormal T2* was not significantly associated with any of the measures of transfusion or chelation. Ferritin levels showed a strong association with LIC. Non-transferrin-bound iron was high in the thalassemia and MDS groups but low in the SCA group (P<0.001). Hepcidin was low in thalassemia, normal in SCA, and markedly elevated in MDS (P<0.001). Two mechanisms may explain that iron deposition largely spares the heart in SCA: the high level of erythropoiesis recycles the iron and the chronic inflammation retains iron within the macrophages. Thalassemia, in contrast, is characterized by inefficient erythropoiesis, unable to handle free iron. Iron accumulation varies widely in MDS syndromes due to the competing influences of abnormal erythropoiesis, excess iron supply, and inflammation.

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Section: Discussioncontrasting

confidence: 99%

Cardiac iron overload in chronically transfused patients with thalassemia, sickle cell anemia, or myelodysplastic syndrome

et al. 2017

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“…compared five children receiving aRBCX with five receiving MET, concluding that for all transfusion events, Hb S control was similar, but for transfusion events less than 40 days apart, aRBCX resulted in lower pretransfusion Hb S . Finally, Fasano et al . and Koehl et al .…”

Section: Discussionmentioning

confidence: 92%

Increased complications of chronic erythrocytapheresis compared with manual exchange transfusions in children and adolescents with sickle cell disease

Woods

Hayashi

Binkley

et al. 2017

Pediatric Blood & Cancer

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“…Manual exchange is easier, does not require much training and can be performed at the patient's bedside. Manual exchange are not as effective as automated exchange in reducing the HbS percentage but can still achieve this if the procedure is performed regularly, [17] though with a higher risk of iron overload than automated exchange. Automated EBT is a faster and neater procedure which takes approximately 2 hours but requires sophisticated machines and trained health personnel.…”

Section: Discussionmentioning

confidence: 99%

Physicians’ Opinion on the Practice of Exchange Blood Transfusion for Treatment of Stroke in Sickle Cell Anaemia

Madu¹

2018

HIJ

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Effectiveness of red blood cell exchange, partial manual exchange, and simple transfusion concurrently with iron chelation therapy in reducing iron overload in chronically transfused sickle cell anemia patients

Abstract: We recommend RCE plus chelation as an effective method for reducing iron overload, while maintaining HbS at 30% to 35%.

Cited by 45 publications

References 31 publications

Cardiac iron overload in chronically transfused patients with thalassemia, sickle cell anemia, or myelodysplastic syndrome

Cardiac iron overload in chronically transfused patients with thalassemia, sickle cell anemia, or myelodysplastic syndrome

Increased complications of chronic erythrocytapheresis compared with manual exchange transfusions in children and adolescents with sickle cell disease

Physicians’ Opinion on the Practice of Exchange Blood Transfusion for Treatment of Stroke in Sickle Cell Anaemia

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