Lack of Cardiac Iron in SCD Patients Despite Severe Iron Overload

Blood ◽  
2014 ◽  
Vol 124 (21) ◽  
pp. 4943-4943
Author(s):  
Rasha I Ahmad ◽  
Sara Keyrouz ◽  
Mariam Arabi ◽  
Fadi Bitar ◽  
Wael al Jaroudi ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Iron Overload ◽  
Sickle Cell ◽  
Cardiac Involvement ◽  
Dry Weight ◽  
Iron Deposit ◽  
Acute Chest Syndrome ◽  
Cell Disease ◽  
Cardiac Iron ◽  
Male Sibling

Abstract In sickle cell disease (SCD), transfusions improve blood flow by reducing the proportion of red cells capable of forming sickle hemoglobin polymer. The major and unavoidable complication of transfusions in SCD is iron overload. Patients with significant transfusion load and iron siderosis, often have iron deposit into multi organs, including liver, pancreas and heart. However, patients with SCD may be relatively protected from iron mediated cardiac toxicity as compared to patients with thalassemia and similar transfusion load. Nonetheless while patients with SCD have less iron deposition in the heart it is presumed that severe loading will eventually lead to cardiac involvement. We report here two patients with scd with severe iron overload who had no cardiac involvement. These are an 18 year old female and her 20 year old male sibling, known to have SCD (HbSS) with significant blood transfusions history, once a month or every two weeks for 12 years duration. Both had undergone splenectomy, and multiple hospitalizations for pain crises and acute chest syndrome. They had received intermittent iron chelation therapy for only 2 years. Examination revealed marked hepatomegaly and highly elevated ferritin levels, 11964ng/ml and 7098ng/ml respectively, suggestive of iron overload. Both patients had unremarkable electrocardiogram and echocardiogram. SGPT and SGOT are normal. Both patients are below the 5thpercentile for height, and l the 18 year old girl is pre-pubertal, has growth hormone deficiency and is on treatment. Cardiac, liver and pancreas magnetic resonance imaging with T2* showed normal cardiac structure and function without siderosis ( heart T2*32 ms and 35 ms in the female and male siblings, respectively [normal >20 ms]). There was however considerable liver siderosis with estimated liver iron content 16.6±4.4 mg/g dry weight (female sibling) and 13.9±1.8 mg/g dry weight (male sibling). The corresponding R2* were 646±165 HZ and 541±62 Hz, respectively. Also, there was mild to moderate pancreatic siderosis in the 18 year old female (R2* 111 HZ) and mild pancreatic siderosis in her sibling (R2* 55 Hz) These two cases while consistent with what has been reported are unusual because of the degree of iron ovreloead. It is an unexepected finding that despite such extensive iron deposits in the liver, there is no evidence of cardiac iron. It thus seems that in patients with sickle cell disease, even with extensive liver siderosis and endocrine dysfunction there is sparing of the heart. Collecting and studying such cases may shed light on the mecahins of cardiac protection from iron overload in sickle cell disease. Disclosures No relevant conflicts of interest to declare.

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.

Outcomes of Chronic Automated RBC Exchange Program in Omaha, Nebraska 2015-2020

Blood ◽  
2020 ◽  
Vol 136 (Supplement 1) ◽  
pp. 21-22
Author(s):  
Aleh Bobr ◽  
Scott A Koepsell ◽  
Julie Eclov ◽  
Omar Abughanimeh ◽  
Steven Ebers ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Blood Cell ◽  
Iron Overload ◽  
Sickle Cell ◽  
Red Blood Cell ◽  
Beta Thalassemia ◽  
Acute Chest Syndrome ◽  
Cell Disease ◽  
Before And After ◽  
Ed Visits

Background: Red blood cell exchange (RBCX) is an effective therapy in the treatment of different hemoglobinopathies. The University of Nebraska Medical Center (UNMC) established a chronic RBCX program in November 2015, which took care of patients with multiple hemoglobinopathies. In this study, we aim to evaluate the outcomes of this program. Methods: This is a retrospective study. After an IRB approval, we reviewed the charts of patients who were enrolled in the chronic RBCX program between 11/2015-7/2020 at UNMC. Data was collected to evaluate indications of RBCX, types of hemoglobinopathies, hemoglobinopathies' complications before and after the enrollment in the program, and assessment of hospital visits before and after enrollment in the program. Results: In November 2015, the chronic RBCX program was established in Nebraska. Since the start, 24 patients came through the program and 20 patients are still actively enrolled and undergoing regular exchange transfusions. The four patients who left the program did it for the following reasons: moving out of state, stem cell transplant and change to different treatment modality. Four of 24 patients were beta thalassemia patients (two of them with combined HbE/beta thalassemia). Twenty patients had sickle cell disease with two of them having combined beta thalassemia and HbS and one with alpha thalassemia and HbS. The indications ranged from history of stroke, intracranial vascular stenosis, acute chest syndrome (ACS), iron overload, multiple vascular occlusive crises (VOC) and intolerance of medications with most of the patients having multiple indications from the list above (Figure 1). There are several positive outcomes from being on the program. In the patients who had been on the program for at least one year (n=11), nine started the program with iron overload and all of them had a significant decrease in serum ferritin (average 751 ng/mL) with three patients returning to normal range. In the patients who had been in the program at least six months (n=16), 13 patients started with iron overload with five returning to normal range and average decrease in ferritin of 585 ng/mL. Another positive outcome is the number of emergency department (ED) visits for pain crisis. We noted reduction in ED visits in all patients who were in the program for at least six months (n=14), with the exception of one patient where the visits were likely the part of drug seeking behavior. In fact 12 of 13 patients had one or no ED visits within one year after starting on the chronic exchange program having had from 2-11 visits a year prior. None of the patients in the program experienced more severe complications of sickle cell disease, like stroke and acute chest syndrome, while on the program. Due to high volumes of transfusion, there is a big concern about developing red blood cell antibodies in sickle cell disease patients who in general have higher red blood cell antibody burden. Out of 24 patients in the program, six had pre-existing antibodies. For the duration of the program, no new alloantibodies were discovered in the chronically exchanged patients despite high transfusion volumes (range 14L-30L/year). The transfused blood was matched for Rh and Kell antigens for the patients with no antibody history. The patients with previous antibody history had additional matching for the antigen to which antibody was directed. Conclusion:Automated chronic RBCX transfusion program is safe to perform. It leads to significant reduction in volume overload and ED visits. Performing high volume transfusions outside of acute sickle cell crisis and with Rh and Kell matched units prevents formation of RBC antibodies Disclosures Gundabolu: BioMarin:Consultancy;Bristol Myers Squibb pharmaceuticals:Consultancy.

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 Iron Overload in Sickle Cell Disease

2010 ◽  
Vol 2010 ◽  
pp. 1-9 ◽  
Author(s):  
Radha Raghupathy ◽  
Deepa Manwani ◽  
Jane A. Little
Keyword(s):  
Sickle Cell Disease ◽  
Iron Overload ◽  
Sickle Cell ◽  
Organ Damage ◽  
Endothelial Damage ◽  
Red Cells ◽  
Acute Chest Syndrome ◽  
Cell Disease ◽  
Treatment And Prevention ◽  
Oxygen Carrying Capacity

In sickle cell disease transfusions improve blood flow by reducing the proportion of red cells capable of forming sickle hemoglobin polymer. This limits hemolysis and the endothelial damage that result from high proportions of sickle polymer-containing red cells. Additionally, transfusions are used to increase blood oxygen carrying capacity in sickle cell patients with severe chronic anemia or with severe anemic episodes. Transfusion is well-defined as prophylaxis (stroke) and as therapy (acute chest syndrome and stroke) for major complications of sickle cell disease and has been instituted, based on less conclusive data, for a range of additional complications, such as priapism, vaso-occlusive crises, leg ulcers, pulmonary hypertension, and during complicated pregnancies. The major and unavoidable complication of transfusions in sickle cell disease is iron overload. This paper provides an overview of normal iron metabolism, iron overload in transfused patients with sickle cell disease, patterns of end organ damage, diagnosis, treatment, and prevention of iron overload.

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 Retrospective Analysis of Compliance and Efficacy of a Novel Formulation of Deferasirox

Blood ◽  
2018 ◽  
Vol 132 (Supplement 1) ◽  
pp. 5877-5877 ◽  
Author(s):  
Jamila Holloway ◽  
Chisom Okezue ◽  
Jennifer Webb
Keyword(s):  
Quality Of Life ◽  
Side Effects ◽  
Sickle Cell Disease ◽  
Iron Overload ◽  
Sickle Cell ◽  
Dry Weight ◽  
Cell Disease ◽  
Transfusion Therapy ◽  
Quality Of Life Measures

Abstract Background: Transfusion-related iron overload is a complication of chronic transfusion therapy in patients with sickle cell disease. Iron overload can cause hepatic, cardiac and other end organ dysfunction, and greater iron burden has been associated with increased mortality in this population. Several medications are available to chelate iron, and adherence to chelation medication is critical to prevent the iron related damage. Jadenu®, a novel film-coated tablet formulation of deferasirox, was introduced in 2015. We assessed the impact of this formulation on adherence, iron control, and patient/parent reported preferences and quality of life in our chronically transfused patients with sickle cell disease. Methods: Patients with sickle cell disease receiving chronic transfusion therapy and chelation were invited to participate in this single-institution trial. Subjects and parents were administered a survey on medication preference and self-reported adherence. Subjects and parents completed the PedsQL™ Sickle Cell Disease Module 3.0 (acute and one month), as well as the PedsQL™ Quality of Life Short Form 4.0 (acute and one month). Retrospective measures of iron burden including laboratory values and imaging was abstracted from the electronic medical record. In subjects who transitioned to tablet deferasirox, iron measures were compared during the time period on their prior chelation and while they were taking tablet deferasirox. Unpaired and paired t-tests were used to compare continuous variables as appropriate. Fisher's exact testing was used to compare categorical data. Results: Twenty one subjects were enrolled in this study. Average age was 15yo (range 8-22yo). At the time of enrollment, 15 subjects were prescribed tablet deferasirox, and six were prescribed deferasirox for oral-suspension (dissolvable). Of those on tablet deferasirox, 92% reported missing more doses with the dissolvable formulation than with the tablet, with 50% reporting missing 3-4 doses per week of the dissolvable formulation. Participants reported barriers to taking the dissolvable formulation included: side effects, need to be taken on an empty stomach, taste, forgetfulness, and general dislike. The majority of subjects (64%) reported no side effects from either formulation. Cost did not appear to be a barrier to taking or obtaining either formulation. There were no statistically significant differences in quality of life measures between subjects taking the two formulations of deferasirox, except patient-reported psychosocial quality of life was higher in 8-13y cohort of subjects taking tablet deferasirox (70.0 vs 86.6, p=0.05). In general, parent-reports of quality of life measures were lower than patient-reports for both groups. For subjects who were on both formulations (n=10), average ferritin and liver iron concentration (LIC) were compared. Average ferritin was comparable during the time periods on dissolvable vs tablet (3358ng/dL vs 3395ng/dL, p>0.05), but there was a trend towards improved LIC on the tablet formulation (15.6mg/g dry weight vs 14.9mg/g dry weight, p>0.05). Discussion: Film-coated tablets were the patient preferred formulation of deferasirox and subjects reported improved adherence with this formulation. Though chelation had little impact on general and sickle cell specific measures of quality of life, there was a trend towards improved iron burden as measured by LIC. Long term evaluations of chelation adherence and impact of iron burden on mortality are needed in patients with sickle cell disease receiving chronic transfusion therapy. 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.

Transfusion therapy for sickle cell disease: a balancing act

Hematology ◽  
2013 ◽  
Vol 2013 (1) ◽  
pp. 439-446 ◽  
Author(s):  
Stella T. Chou
Keyword(s):  
Sickle Cell Disease ◽  
Iron Overload ◽  
Sickle Cell ◽  
Perioperative Complications ◽  
Iron Chelation ◽  
Acute Chest Syndrome ◽  
Cell Disease ◽  
Transfusion Therapy ◽  
Oral Agents ◽  
Blood Group Typing

AbstractTransfusion therapy is a key intervention in decreasing morbidity and mortality in patients with sickle cell disease (SCD). Current indications for acute and chronic transfusion therapy have significantly increased the number of RBC units transfused to patients with SCD worldwide. This review summarizes transfusion management for the treatment or prevention of neurologic and perioperative complications, acute chest syndrome, and acute anemia associated with SCD. Despite the recognized benefits of transfusion therapy, it is not without the risks of iron overload, alloimmunization, and delayed hemolytic transfusion reactions. Transfusional iron overload management includes automated RBC exchange, noninvasive imaging to monitor iron burden, and iron chelation with parenteral or oral agents. Although limited and extended RBC antigen matching reduces antibody formation, the prevalence of RBC alloimmunization in patients with SCD remains high. Recent studies demonstrate that RH genetic diversity in patients with SCD contributes to Rh alloimmunization, suggesting that even more refined RBC matching strategies are needed. Advances in molecular blood group typing offer new opportunities to improve RBC matching of donors and recipients and can be of particular benefit to patients with SCD.

scholarly journals The relationships between pancreatic T2* values and pancreatic iron loading with cardiac dysfunctions,  hepatic and cardiac iron siderosis among Egyptian children and young adults with β-thalassaemia major and sickle cell disease: a cross-sectional study

F1000Research ◽  
2021 ◽  
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 ◽  
Iron Loading ◽  
Thalassaemia Major ◽  
Cell Disease ◽  
Cardiac Iron ◽  
Diastolic Velocity

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 assess the relationships between pancreatic T2* values and pancreatic iron loading with cardiac dysfunctions and liver and cardiac 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 and sex-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 mean age of our patients was 13.7 years with 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 peak late diastolic velocity at septal mitral annulus (r=0.269, p=0.038), peak early diastolic velocity at tricuspid annulus (r=0.430, p=0.001) and mitral annular plane systolic excursion (r=0.326, p=0.01); and negatively with end systolic pulmonary artery pressure (r=-0.343, p=0.007) and main pulmonary artery diameter (MPA) (r=-0.259, p=0.046). We couldn’t test the predictability of pancreatic T2* in relation to cardiac T2* as only one patient had cardiac T2*<20 ms. Conclusion: There was a relationship between pancreatic iron siderosis with cardiac dysfunction in multi-transfused patients with βTM and SCD. No direct relation between pancreatic iron and cardiac siderosis was detected.

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