MRI Evidence of Cardiac Iron Accumulation in Myelodysplasia and Unusual Anaemias.

Blood ◽  
2006 ◽  
Vol 108 (11) ◽  
pp. 1553-1553 ◽  
Author(s):  
Julie Glanville ◽  
Perla Eleftheriou ◽  
John Porter
Keyword(s):  
Iron Overload ◽  
Sickle Cell ◽  
Sickle Cell Anaemia ◽  
Iron Chelation ◽  
Iron Accumulation ◽  
Thalassaemia Major ◽  
Cardiac Iron ◽  
Sideroblastic Anaemia ◽  
Cardiac Iron Overload ◽  
Thalassaemia Intermedia

Abstract Iron overload is a well described complication of multiple transfusions. Cardiac failure secondary to myocardial iron accumulation is the leading cause of death in thalassaemia major patients, and survival is improved with iron chelation. Identifying patients at risk of complications from iron overload is now more widely available with the MRI T2* technique. Iron chelation improves survival in thalassaemia major patients, but the significant practical difficulties limit its use in acquired anaemias. It has recently been suggested that abnormal cardiac T2* values do not occur in multitransfused elderly patients with aquired sideroblastic anaemia (RARS) (Winder et al Blood 2005 106: Abstract 2536). Here we examine the frequency of cardiac iron accumulation as evidenced by a shortening of the myocardial T2* value in multi transfused patients with myelodysplasia (MDS), sickle cell anaemia, and other transfusion dependent anaemias, including diamond blackfan anaemia (DBA) and pyruvate kinase deficiency (PKD). Method: Database records of cardiac magnetic resonance T2* values were assessed on 41 non sickle, non thalassaemia patients, 131 thalassaemia major and intermedia and 37 sickle cell patients, where a shortening of the cardiac T2* value below 20ms is associated with iron overload. 7 patients with MDS were assesed for transfusion duration and intensity, iron chelation and hepatic T2* values. Results: 14 out of 41 (34%) patients with non-sickle, non-thalassaemia transfusion dependent anaemia had abnormal cardiac T2* values, compared with 48% of thalassaemia major patients,13% thalassaemia intermedia and 2.7% sickle cell anaemia Table1. Table 1: Percentage of patients with Cardiac iron overload as evidenced by MRI T2* <20ms in unusual anaemias, thalassaemias and sickle cell anaemia DIAGNOSIS NO. PATIENTS AVERAGE AGE (Yrs) NO. PATIENTS WITH T2*<20 ms Hb H 2 40.5 (35–46) O Haemolytic Anaemia Unknown Cause 1 11 0 Osteopetrosis 1 49 0 AML/BMT/MF 5 26.4 (6–49) 1 (20%) Myelodysplasia 1 41 0 CDA 1 41 0 Erythropoietic Porphyria 1 40 0 Red Cell Aplasia 2 36 (35–37) 0 DBA 7 25 (9–36) 5 (71%) PKD 9 26.2 (14–47) 2 (22%) Congenital Sideroblastic Anaemia 4 35.3 (22–64) 3 (75%) Thalassaemia major 108 26 (1– 51) 52 (48%) Thalassaemia intermedia 23 34.6 (14–60) 3 (13%) Sickle Cell Anaemia 37 37.5 (21–58) 1 (2.7%) In patients with MDS, cardiac iron overload occurs between 2 and 4 years of consistent transfusion, but may not occur even after 12 years. Transfusion intensity but not total blood volume is higher in those with cardiac iron load. Hepatic iron overload is more severe in those with cardiac iron overload, and ferritin values are higher (Average 5865 ug/l v 2832 ug/l) but neither predicts cardiac iron load One out of three patients with T2* under 20ms was heterozygous for C282Y and one heterozygous for H63D.Table2. Table 2: A comparison of transfusion intensity, duration, iron chelation and hepatic T2* values in patients with and without cardiac iron overload in myelodysplasia MYELODYSPLASIA T2* < 20 T2* > 20 Number of Patients 3 4 Average Years of Transfusion (yrs) 208 204 Average No of Red Cell Units Transfused 3.3 (2–4) 6.1 (2–12) Average Hepatic T2*(ms) 1.4 (1.2–1.6) 4.7 (1.2–14.7) Proportion on iron chelation 1/3 2/4 Average Units/Year 51.3 40.1 Conclusion: Iron accumulation can occur in elderly patients with myelodysplasia after only two years of transfusion. Early consideration of iron chelation is appropriate and additional risk factors, eg inheritance of HFE gene mutations should be determined.

Assessment of Cardiac Iron Overload in Chonically Transfused Patients with Thalassemia, Sickle Cell Anemia, and Myelodysplastic Syndromes

Blood ◽  
2015 ◽  
Vol 126 (23) ◽  
pp. 2151-2151
Author(s):  
Mariane De Montalembert ◽  
Jean-Antoine Ribeil ◽  
Valentine Brousse ◽  
Agnes Guerci-Bresler ◽  
Aspasia Stamatoullas ◽  
...  
Keyword(s):  
Iron Overload ◽  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Iron Chelation ◽  
Iron Level ◽  
Normal Range ◽  
Liver Iron ◽  
Cardiac Iron ◽  
Cardiac Iron Overload ◽  
Massive Outflow

Abstract Background. Cardiac iron overload is the major cause for death in regularly transfused thalassemic (Thal) patients. Its impact in myelodysplastic syndrome (MDS) patients is debated. Heart seems to be spared in regularly transfused patients with sickle cell anemia (SCA), which is supposed to be related to a later onset on transfusions and to the use of erythrocytapheresis rather than simple transfusion. Our aim was to assess the prevalence of cardiac iron overload, defined as a T2*cardiovascular magnetic resonance (MR) <20ms, and to look for predisposing factors. Patients and methods. Patients were enrolled if they were regularly followed in a center where the exact number of erythrocyte concentrates (EC) could be obtained, had received in the previous year more than 8 EC, were older than 6 years (limit for MRI without sedation), had no known heart disease related to another pathology, and had given informed consent. All patients underwent 1.5T myocardial T2*MR imaging after validation of the procedures in the different sites. Assessment of Liver Iron Content (LIC) used two signal intensity ratio of gradient echo imaging (R2*) MRI protocols. Serum Non-Transferrin Bound Iron (NTBI) was measured by the FeROSTM eLPI kit, and serum hepcidin by LC-MSMS. Results. 20 Thal, 41 SCA and 25 MDS patients were evaluable. We divided SCA in 2 groups, according to the procedure recorded at the time of the study, manual exchange transfusion (G1, N = 30 patients), or erythrocytapheresis (G2, N=11 patients). We found cardiac overload in 0, 3 (15%), and 4 (16%) of SCA, Thal, and MDS patients respectively. Serum ferritins at beginning of chelation were not statistically different in all categories of patients, as well as Ferritin and LIC at the time of the study. Increased LIC and abnormal T2* were associated in Thal and MDS patients (p=0.04), with no correlation between abnormal T2* and parameters of transfusion and chelation. Plasmatic iron level was increased in Thal and MDS patients but remained at normal range in SCA patients. NTBI level was high in Thal and MDS but completely absent in SCA groups. The major discrepancy was in the values of hepcidin, which were collapsed in Thal, at normal range in SCA, and highly elevated in MDS patients. Discussion and conclusion. We confirm that SCA patients are relatively protected from cardiac iron overload. This results probably from massive consummation of iron through effective erythropoiesis, making toxic free iron (NTBI) less available in the circulation. In addition, since iron overload in SCA results from a massive outflow of hemoglobin (Hb) due to intravascular hemolysis and transfusion, the heme/Hb-bound iron must be efficiently handled in liver macrophages, limiting its release in the bloodstream. In Thal patients, underlying defects in erythropoietic processes, together with low hepcidin that stimulates intestinal iron absorption and increases NTBI, must provoke more organ damages. Hepcidin levels were high in MDS patients, suggesting that transfusion-dependent iron overload was a more effective regulator of hepcidin production than dyserythropoiesis. The % of T2*<20 ms we observed in MDS patients (16%) was quite comparable with previous publications. Finally, we observe that, in opposition with previous reports, SCA patients undergoing eythrocytapheresis may experience severe iron overload and need iron chelation. Table 1. Thal SCA G1 SCA G2 MDS p Age at beginning of transfusion (yrs) 8.5[0-45] 7[0-45] 16.5[1-55] 66[38-83] <0.001 Duration of transfusion (yrs) 10[1-39] 7[1-22] 10.5[0-25] 3[1-10] <0.001 N EC since diagnosis 359[21-1360] 139[24-791] 201[14-888] 77[16-544] 0.0005 N CE/yr 24[8-67] 21[4-62] 35[17-58] 27[7-65] 0.09G1vsG2=0.03 % patientschelated 95 90 72.7 72 0.12 Age at beginning of chelation (yrs) 11[1-48] 9[2-47] 18[6-31] 68[38-84] <0.0005 Ferritin at beginning of chelation (ng/ml) 1148[713-2400] 2075[448-3670] 1500[905-2804] 2398[482-5140] 0.22 N T2*<20 ms 3(15%) 0 0 4(16%) 0.01 LIC (mg/g d.w.) 10.4[0.8-20.2] 10.7[0.8-37.1] 14[0.8-19.7] 15.2[3.0-45.3] 0.29 Plasmatic iron(μmol/l) 36.9[31-57] 22.5[6-42.2] 21[13-46] 38.2[11.9-72] <0.001 NTBI (mg/ml) 7.1[0-31.1] 0[0-18.3] 0[0-12.4] 4.45[0-25.5] 0.0005 Ferritin (ng/ml) 870[169-4339] 2739[393-5596] 2404[33-20030] 1611[223-6813] 0.08 Hepcidin (ng/ml) 1.35[0-12.3] 9.95[0-67.9] 2.10[0-52.4] 36.35[3-143.2] <0.001 Deferasirox dosage<0.5 μg/ml 3/8(38%) 3/10(29%) 3/5(60%) 0/11(0%) 0.03 Disclosures De Montalembert: Addmedica: Membership on an entity's Board of Directors or advisory committees; Novartis: Speakers Bureau. Guerci-Bresler:ARIAD: Speakers Bureau; BMS: Speakers Bureau; Novartis: Speakers Bureau; PFIZER: Speakers Bureau.

scholarly journals Plasma level of matrix metalloproteinase-9 in patients with sickle cell disease and its correlation to myocardial iron overload

2020 ◽  
Author(s):  
Tamer Hassan ◽  
Mohamed Badr ◽  
Mohamed Arafa ◽  
Doaa Abdel Rahman ◽  
Manar Fathy ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Iron Overload ◽  
Sickle Cell ◽  
Serum Ferritin ◽  
Plasma Levels ◽  
Restrictive Cardiomyopathy ◽  
Myocardial Iron ◽  
Cell Disease ◽  
Cardiac Iron ◽  
Cardiac Iron Overload

Abstract Cardiac iron overload is secondary to chronic blood transfusion in patients with sickle cell disease (SCD). Iron overload cardiomyopathy is a restrictive cardiomyopathy associated with systolic and diastolic dysfunction. Matrix metalloproteinases (MMPs) are a family of zinc-dependent endopeptidases responsible for tissue remodeling. Many studies offer strong evidence for the role of MMP-9 in LV remodeling. We aimed to detect plasma levels of MMP-9 in patients with SCD and its correlation to myocardial iron overload. A case control study was carried out on 50 patients with SCD and 50 age and sex matched healthy controls. Assessment of cardiac iron overload in patients by MRI T2* was performed. Plasma MMP-9 levels were measured for patients and controls using ELISA. SCD patients had significantly higher levels of MMP-9 than controls. There was highly significant correlation between plasma levels of MMP-9 and serum ferritin. Patients with vaso-occlusive crises (VOC) > 5/year had significantly higher levels of MMP-9 than those with VOC ≤ 5 /year. No significant correlation was found between MMP-9 and cardiac T2*. MMP-9 seems to be a useful marker in SCD patients. Patients with serum ferritin > 1000 ng/ml, recurrent VOC > 5 /year had significantly higher MMP-9 serum levels than others.

Cardiac Iron Overload Causes Clinically Evident Heart Failure and Arrhythmia in Sickle Cell Anemia Patients: Evidence From Three Cases

Blood ◽  
2011 ◽  
Vol 118 (21) ◽  
pp. 4846-4846
Author(s):  
Bhakti P. Mehta ◽  
Vasilios Berdoukas ◽  
Mammen Puliyel ◽  
Adam Bush ◽  
Thomas Hofstra ◽  
...  
Keyword(s):  
Heart Failure ◽  
Iron Overload ◽  
Sickle Cell ◽  
Research Funding ◽  
Chelation Therapy ◽  
Liver Iron ◽  
Cardiac Iron ◽  
Cardiac Iron Overload ◽  
Clinical Heart Failure ◽  
Symptoms And Signs

Abstract Abstract 4846 Transfusional iron overload is associated with poor outcomes in sickle cell disease (SCD). Unlike in thalassemia major (TM), there is no evidence that the iron overload per se causes morbidity in SCD. We present two patients with clear evidence of heart failure and arrhythmia secondary to transfusion induced cardiac iron overload, whose symptoms and signs completely resolved after a short period of intensive iron chelation. We studied 134 patients with SCD with magnetic resonance imaging (MRI). Over 50% of patients with TM and 70% of patients with transfusion dependent Diamond Blackfan Anemia demonstrate cardiac iron overload. We reviewed 472 MRIs in 134 patients with SCD. The median liver iron concentration (LIC) was 10.2 mg/g dry weight (dw). Ten percent of the patients had liver iron > 35mg/g dw. Three (2.2%) demonstrated cardiac iron overload. Patient 1 is now 27 years old and began transfusions at the age of 15 years because of pulmonary hypertension. The first MRI performed at the age of 22 years showed LIC >50 mg/g dw and a cardiac R2* of 128 sec−1 (T2* 7.8 ms) that indicates severe cardiac iron load. At this time she was changed from deferasirox to continuous infusion of desferrioxamine. After 6 months the LIC was 47 mg/g dw and her cardiac R2* was 123sec−1 (T2* 8.1ms). She had dyspnea on mild exertion, ankle edema, and orthopnea. Her left ventricular ejection fraction (LVEF) by MRI at that time was 45%. She started intensive chelation therapy with deferiprone (on compassionate basis) 100mg/kg/day and deferasirox 40mg/kg/day. Her symptoms and signs of clinical heart failure resolved within two months. She remains asymptomatic. After 7 months cardiac R2* is 88 sec−1 (T2*11.3ms) with an LVEF of 55% and LIC of 36 mg/g dw. Patient 2 is now 32 years of age. She started regular blood transfusions at the age of 9 years. Her first MRI at the age of 27 years showed a LIC of >60 mg/g dw and no evidence of cardiac iron overload with a cardiac R2* of 29 sec−1(T2* 34.9ms) with an LVEF of 61%. After 2.5 years her cardiac R2* was 68 sec−1 (T2* 14.7 ms) with an LVEF of 65.7% and 18 months later it was 123 sec−1(T2* 8.1 ms) with an LVEF of 72%. She developed significant arrhythmias coincident with her rapid cardiac iron loading. She was started on compassionate use deferiprone and deferoxamine, with which she is poorly compliant. Repeat cardiac MRI showed a worsening of cardiac iron with R2* of 204 sec−1 (T2* 4.9ms) after 8 months with an improved LVEF of 72%. She currently continues of her regular transfusions and deferiprone and is awaiting repeat MRI. Her LVEF improved while on the chelation therapy despite the deterioration in her cardiac iron content. This is consistent with our observation that LVEF tends to improve even with intermittent chelation although the cardiac iron may not decrease. Patient 3 died of numerous complications of SCD at the age of 19 years. She had started transfusions at the age of 10 years, because of a cerebrovascular accident. At the age of 14 years her first abdominal MRI demonstrated a LIC of 12.8 mg/g dw. She had her first cardiac MRI at the age of 16 years which showed no evidence of cardiac iron with a R2* of 30 sec−1 (T2* 32.7ms), which worsened to 57 (T2* 17.4ms) at the age of 17, reflecting a small but rapid increase in cardiac iron. Patient 1 and 2 demonstrate that transfusional iron overload can directly cause life threatening complications in patients with SCD. Patient 1 in particular, was in overt clinical heart failure that responded dramatically to intensification of chelation therapy. These data underscore the importance of direct measurement of tissue iron concentrations and points out that though uncommon, cardiac iron overload can occur in patients with sickle cell anemia with serious consequences. Disclosures: Berdoukas: ApoPharma Inc.: Consultancy. Carson:ApoPharma Inc.: Honoraria; Novartis Inc: Speakers Bureau. Wood:Cooleys Anemia Foundation: Honoraria, Membership on an entity's Board of Directors or advisory committees, Research Funding; Ferrokin Biosciences: Consultancy; Novartis: Research Funding. Coates:Novartis Inc: Speakers Bureau.

Cardiac Iron Overload In Sickle-Cell Disease

Blood ◽  
2013 ◽  
Vol 122 (21) ◽  
pp. 1013-1013
Author(s):  
Antonella Meloni ◽  
Mammen Puliyel ◽  
Alessia Pepe ◽  
Massimo Lombardi ◽  
Vasilios Berdoukas ◽  
...  
Keyword(s):  
Myocardial Infarction ◽  
Sickle Cell Disease ◽  
Iron Overload ◽  
Sickle Cell ◽  
Clinical Characteristics ◽  
Transferrin Saturation ◽  
Thalassemia Major ◽  
Cell Disease ◽  
Cardiac Iron ◽  
Cardiac Iron Overload

Abstract Introduction Chronically transfused sickle cell disease (SCD) patients have lower risk of endocrine and cardiac iron overload load than comparably transfused thalassemia major patients. The mechanisms for this protection remain controversial but likely reflects lower transferrin saturation and circulating labile iron pools because of chronic inflammation and regeneration of apotransferrin through erythropoiesis. However, cardioprotection is incomplete; we have identified 6 patients out of the 201 patients (3%) followed at our Institution who have prospectively developed cardiac iron. We present the clinical characteristics of these patients to identify potential risk factors for cardiac iron accumulation. Methods Cardiac, hepatic, and pancreatic iron overload were assessed by R2* Magnetic Resonance Imaging (MRI) techniques as extensively described by our laboratory. The medical records of the selected patients were reviewed for demographic data, for transfusion and chelation history and for hematologic and biochemical parameters. Results Table 1 describes clinical characteristics of the six patients at the time they developed detectable cardiac iron (R2* ≥ 50 ms). Patient 6 was included because he showed a R2* of 49 Hz that was increasing rapidly. Five of the six patients were managed on simple transfusions. Five patients had been on chronic transfusion for more than 11 years. The three patients who developed cardiac iron the earliest (3.7 – 14 years of transfusions) had more efficient suppression of endogenous red cell production (HbS levels 2-5%) compared with patients who required longer transfusional exposure (HbS levels 13.3 – 41%). All patients had qualitatively poor chelation compliance (<50%), based upon their prescription refill rate. All patients had serum ferritin levels exceeding 4600 and liver iron concentration (LIC) greater than 22 mg/g. Pancreatic R2* was greater than 100 Hz in every patient studied (5/6). Figure 1 shows the longitudinal relationship between iron overload in the heart and in the other organs for each patient; initial iron levels are shown in black. Cardiac R2* appears increase dramatically once a critical LIC “threshold” is reached, qualitatively similar to the 18 mg/g threshold observed in thalassemia major patients. Cardiac R2* rose proportionally to pancreas R2*, similar to thalassemia major patients, with all of the patients having pancreas R2* > 100 Hz at the time cardiac iron was detected. Conclusions Cardiac iron overload occurs in a small percentage of chronically transfused SCD patients and is only associated with exceptionally poor control of total body iron stores. Duration of chronic transfusion is clearly important but other factors, such as levels of effective erythropoiesis, may also contribute to cardiac risk. The relationship between cardiac iron and pancreas R2* suggests that pancreas R2* can serve as a valuable screening tool for cardiac iron in SCD patients. Disclosures: Berdoukas: ApoPharma inc: Consultancy. Coates:ApoPharma inc, Novartis, Shire: Consultancy. Wood:Novartis: Consultancy, Honoraria; Shire: Consultancy, Research Funding; ApoPharma: Consultancy, Honoraria, Use of deferiprone in myocardial infarction, Use of deferiprone in myocardial infarction Patents & Royalties.

scholarly journals Cardiac Iron Overload in Sickle Cell Disease: When to Screen and How to Intervene

Blood ◽  
2018 ◽  
Vol 132 (Supplement 1) ◽  
pp. 528-528
Author(s):  
Amy Y Tang ◽  
Cassandra D Josephson ◽  
Kristina Lai ◽  
Peter A. Lane ◽  
Ross M. Fasano
Keyword(s):  
Risk Factors ◽  
Sickle Cell Disease ◽  
Iron Overload ◽  
Sickle Cell ◽  
Clinical Characteristics ◽  
Iron Loading ◽  
Cell Disease ◽  
Transfusion Therapy ◽  
Cardiac Iron ◽  
Cardiac Iron Overload

Abstract Background Iron overload is a recognized consequence of chronic transfusion therapy in patients with sickle cell disease (SCD), but most of the focus to date has been on the effects of increased liver iron concentration (LIC) with increasing transfusion burden. Even though there is a robust body of literature concerning cardiac iron overload (CIO) in patients with thalassemia major, there remains a paucity of data in how to detect and treat CIO in patients with SCD, particularly in the pediatric and young adult population. While CIO is seen less commonly in sickle cell disease than in thalassemia, patients with SCD remain at risk, with recent studies demonstrating an incidence of 2-5% of CIO in chronically transfused patients with SCD. We performed a retrospective chart review of patients with cardiac MRIs (cMRIs) and LICs by Ferriscan performed at our institution to identify risk factors for CIO, as well as to characterize institutional practice for assessing cardiac iron in the absence of defined practice guidelines. Methods We reviewed clinical characteristics of all patients with SCD who had cMRIs performed at Children's Healthcare of Atlanta between June 2012 and December 2017. We then queried our institutional sickle cell database for patients who were at least 3 years old in 2010, genotype SS or S Beta zero thalassemia, were on chronic transfusions for at least 5 years by 2017, and had not undergone a cMRI. Patients who were status post bone marrow transplant were excluded. For comparison of age, average ferritin, and transfusion duration, significance among means between patients with and without CIO was calculated using a two-tailed unpaired t-test. For comparison of LIC, significance among medians was calculated using the Mann Whitney test. A p value of <0.05 was considered significant. Statistical analyses were performed using Prism 6 (GraphPad Software, Inc.). Results Of 36 evaluable patients who had undergone cMRI, there were 11 with CIO, as defined by a T2* < 20ms. Clinical characteristics are shown in Figure 1. Patients were 7-28 years of age, and had received chronic transfusion therapy for a range of 22 months to 228 months. Between patients who did and did not have CIO, there was no significant difference in average 1-year ferritin level (6786 vs 6373 ng/mL, p=0.79), transfusion duration (103 vs 123 months, p=0.41), or age (15 vs 18 years, p=0.12). There was a higher median LIC by Ferriscan of > 43 mg/g in those with CIO vs 34 mg/g in those without CIO, although this was not statistically significant (Figure 1). Interestingly, CIO was seen as young as 7 years of age and after as little as 22 months of chronic transfusions, and with concurrent LIC values as low as 8.1 mg/g. Of the 11 patients with CIO, 6 had follow-up cMRI data available, and all 6 had normalization of cardiac iron (T2* > 20ms) on subsequent MRIs (Figure 2 and Table 2). There was 1 patient who did not have full transfusion and chelation history available for analysis. Of the remaining 5, 5/5 had increased or more aggressive chelation added, including 2 who were started on high-dose IV Desferal every 2 weeks; 3/5 also had partial manual exchange (PME) added to their chronic transfusion regimens. There were 80 patients who were on chronic transfusions but did not have a cMRI performed; as a group, they had a median LIC of 17 mg/g (range: 1.7 - >43 mg/g), an average 1-year ferritin of 3641 ng/mL (range: 520 - 8478 ng/mL), and had been on chronic transfusions for a mean of 87 months at time of Ferriscan study (range: 14 - 192 months). Overall, these patients had a lower transfusion burden than those who received cMRIs, but there were several in this group who had significant iron overload, including 10 who had LIC values of > 43mg/g. Conclusion CIO in SCD may be a more salient issue, and occur earlier, than previously described. We did not find a strong relationship between CIO and ferritin levels or LIC by Ferriscan, but we did find that CIO was reversible with more aggressive chelation or the addition of PME. While guidelines for monitoring for CIO in SCD are largely extrapolated from thalassemia data, the rate and physiology of iron loading may be completely different. Due to a paucity of information in this area, more studies are needed to guide screening and to fully assess risk factors that may put certain individuals more at risk for cardiac iron loading. Disclosures No relevant conflicts of interest to declare.

scholarly journals Iron overload parameters and early detection of cardiac disease among Egyptian children and young adults with β-thalassaemia major and sickle cell disease: a cross-sectional study

F1000Research ◽  
2020 ◽  
Vol 9 ◽  
pp. 1108
Author(s):  
Khaled Salama ◽  
Amina Abdelsalam ◽  
Hadeel Seif Eldin ◽  
Eman Youness ◽  
Yasmeen Selim ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Pulmonary Artery ◽  
Iron Overload ◽  
Sickle Cell ◽  
Systolic Pulmonary Artery Pressure ◽  
Thalassaemia Major ◽  
Cell Disease ◽  
Cardiac Iron ◽  
Diastolic Velocity ◽  
Egyptian Children

Background: Cardiac, hepatic and pancreatic T2* measured by magnetic resonance imaging (MRI) has been proven to be an accurate and non-invasive method for measuring iron overload in iron overload conditions. There is accumulating evidence that pancreatic iron can predict cardiac iron in young children because the pancreas loads earlier than the heart. The aim of our study was to investigate cardiac function and cardiac iron and their relation to pancreatic iron among patients with β-thalassaemia major (βTM) and sickle cell disease (SCD). Methods: 40 βTM and 20 transfusion-dependant SCD patients were included along with 60 healthy age-matched controls. Echocardiography and Tissue Doppler Imaging were performed for all subjects as well as the control group.  Hepatic, cardiac and pancreatic iron overload in cases were assessed by MRI T2*. Results: The study group consisted of 40 βTM and 20 transfusion dependant SCD patients with mean age 13.7 years and mean frequency of transfusion/year 12. Mean cardiac T2* was 32.9 ms and mean myocardial iron concentration was 0.7 mg/g; One patient had cardiac iron overload of moderate severity. Mean pancreatic T2* was 22.3 ms with 20 patients having mild pancreatic iron overload. Pancreatic T2* correlated positively with main pulmonary artery diameter (p=0.046), peak late diastolic velocity at septal mitral annulus (p=0.038), peak early diastolic velocity at tricuspid annulus (p=0.001) and mitral annular plane systolic excursion (p=0.01); and negatively with end systolic pulmonary artery pressure (p=0.007). We couldn’t test the predictability of pancreatic T2* in relation to cardiac T2* as only one patient had cardiac T2*<20 ms. Conclusion: Assessment of pancreatic T2* in multi-transfused patients with βTM and SCD can predict myocardial dysfunction. No direct relation between pancreatic iron and cardiac siderosis was detected.

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