Plasma level of matrix metalloproteinase-9 in patients with sickle cell disease and its correlation to myocardial iron overload (original) (raw)
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Cardiac iron overload in sickle-cell disease
American Journal of Hematology, 2014
Chronically transfused sickle cell disease (SCD) patients have lower risk of myocardial iron overload (MIO) than comparably transfused thalassemia major (TM) patients. However, cardioprotection is incomplete. We present the clinical characteristics of six patients who have prospectively developed MIO, to identify potential risk factors for cardiac iron accumulation. From 2002 to 2011, cardiac, hepatic, and pancreatic iron overload were assessed by R 2 and R 2 * magnetic resonance imaging techniques in 201 chronic transfused SCD patients as part of their clinical care. At the time, they developed MIO, five of six patients had been on chronic transfusion for more than 11 years; only one was on exchange transfusion. The time to MIO was correlated with reticulocyte and hemoglobin S percentages. All patients had qualitatively poor chelation compliance (<50%). All patients had serum ferritin levels >4600 ng/ml and liver iron concentration >22 mg/g. Pancreatic R 2 * was >100 Hz in every patient studied (5/6). Cardiac iron rose proportionally to pancreas R 2 *, with all patients having pancreas R 2 *>100 Hz when cardiac iron was present. MIO had a threshold relationship with liver iron that was higher than observed in TM patients. In conclusion, MIO 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, appear to contribute to cardiac risk. Pancreas R 2 * can serve as a valuable screening tool for cardiac iron in SCD patients.
Frontiers in Medicine, 2021
The treatment of sickle cell disease (SCD) is mainly supportive, except for a minority, who receive bone marrow transplantation (BMT). Serum ferritin (SF) is routinely available but is notoriously unreliable as a tool for iron-overload assessment since it is an acute-phase reactant. Although blood transfusion is one of the most effective ways to deal with specific acute and chronic complications of SCD, this strategy is often associated with alloimmunization, iron overload, and hemolytic reactions. This study, thus, aims to evaluate iron overload in patients with SCD on chronic blood transfusions and specifically, correlate SF with the current standard of care of iron-overload assessment using MRI-based imaging techniques. Amongst a historic cohort of 58 chronically transfused patients with SCD, we were able to evaluate 44 patients who are currently alive and had multiple follow-up testing. Their mean age (±SD) was 35 (9) years and comprised of 68.2% of women. The studied iron-overl...
Myocardial tissue characterization and the role of chronic anemia in sickle cell cardiomyopathy
Journal of Magnetic Resonance Imaging, 2007
Purpose: To use cardiovascular magnetic resonance (CMR) techniques to examine possible causes for the left ventricular (LV) dilatation that occurs in sickle cell disease (SCD), including the effects of chronic anemia, iron-induced cardiomyopathy, and regional fibrosis due to sludge infarcts that occur during sickle crises. Materials and Methods: A total of 47 patients with sickle cell anemia were assessed for LV function and myocardial iron levels using CMR measurements; 30 of these were also assessed for regional fibrosis using late gadolinium-enhancement CMR. The LV function was compared to both normal controls and transfusion dependent non-ironloaded (NIL) thalassemia major (TM) patients. Results: Only one SCD patient had significant myocardial iron loading, and only two patients had regional fibrosis. There were significant differences in ventricular volumes of the sickle patients compared with both the normal controls and the NIL-TM population (P Ͻ 0.01). Conclusion: The LV changes seen in SCD are partly the result of a chronic anemia but there appears to be another contributory factor. This extra factor is not myocardial iron loading or regional fibrosis, although a homogenous fibrotic disorder affecting the left ventricle cannot be excluded.
Iron status in sickle cell disorders
International Journal of Medical Science and Public Health, 2016
Background: There is a general view that patients with sickle cell disease (SCD) reveal iron overload and that iron deficiency anemia is uncommon in them because of the availability of an adequate iron source from increased red cell turnover and blood transfusions. However, there are reports of iron deficiency in a proportion of these patients, which can often be missed because they exhibit already anemic condition. Objective: To evaluate the iron status and to determine the incidence of iron deficiency in sickle cell disorders including subjects with SCD, sickle cell trait (SCT), and compound heterozygosity. Materials and Methods: A total of 155 subjects were included in study; 138 of these showed homozygous sickle cell anemia (SS) and 17 sickle cell trait (AS). Presence of HbS was detected by solubility test, followed by hemoglobin phenotype determination by automated HPLC. The measurement of serum iron and total iron-binding capacity was done by ferrozine method, serum ferritin by radioimmunoassay, and zinc protoporphyrin (ZPP) by using ProtoFluor-Z Hematofluorimeter, while hematological parameters were determined using a Sysmex Kx21 autoanalyzer. Result: Of the 155 patients studied, 7 (4.5%) showed iron deficiency and 33 (21.3%) showed iron overload. Iron and serum ferritin values in transfused patients were significantly higher than in nontransfused patients. The mean reticulocyte count was significantly lower in iron deficient group than that in iron sufficient group. The ZPP values were elevated in all iron deficient and the 39 of 148 iron sufficient/overloaded subjects; however, ZPP values were much higher in the iron deficiency group. Conclusion: A state of iron deficiency may be present in SCD and SCT patients. Therefore, we recommend that patients with SCD and SCT should be screened for iron deficiency by conventional laboratory tests.
Serum Iron Levels and Copper-to-Zinc Ratio in Sickle Cell Disease
Medicina
Background and Objectives: Altered copper and zinc homeostasis may influence the antioxidant defense system and consequently lead to oxidative stress and associated complications in sickle cell disease (SCD) patients. Iron levels have been reported to increase in sickle cell patients due to frequent blood transfusion, chronic intravenous haemolysis and increased absorption of iron from the gastrointestinal tract. These elevated levels of iron may also lead to extensive oxidative damage. The current study evaluated serum levels of iron, copper and zinc in SCD patients and “healthy” controls. Materials and Methods: The study was a cross-sectional one, comprising 90 SCD patients with Haemoglobin SS and Haemoglobin SC genotypes and 50 HbAA “healthy” controls. Serum levels of iron, copper and zinc were measured using a Flame Atomic Absorption Spectrometer (Variant 240FS manufactured by VARIAN Australia Pty Ltd, VIC, Australia). Copper and zinc ratios were calculated and analyzed. Results...
F1000Research
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/yea...
European Journal of Haematology, 2009
In the past, most patients with sickle cell disease (SCD) were not placed on regular transfusion regimens. However, several recent reports have recommended transfusion therapy for this patient population to prevent primary or recurrent neurovascular complications (1). Although transfusions may help to minimize disease-specific morbidity and mortality in this condition, they are not without their own side effects. In b-thalassemia major patients, chronic transfusion therapy often results in massive iron overload in the liver, heart, and endocrine glands, leading