scholarly journals Analysis of liver iron concentration in an elderly female undergoing hemodialysis with calcific uremic arteriolopathy does not support the role of iron overload in calciphylaxis: lesson for the clinical nephrologist

2021 ◽  
Author(s):  
Guy Rostoker ◽  
Patricia Senet ◽  
Fanny Lepeytre ◽  
Mireille Griuncelli ◽  
Christelle Loridon ◽  
...  
Keyword(s):  
Iron Overload ◽  
Iron Concentration ◽  
Liver Iron Concentration ◽  
Liver Iron ◽  
Elderly Female ◽  
Calcific Uremic Arteriolopathy

Consequences and management of iron overload in sickle cell disease

Hematology ◽  
2013 ◽  
Vol 2013 (1) ◽  
pp. 447-456 ◽  
Author(s):  
John Porter ◽  
Maciej Garbowski
Keyword(s):  
Sickle Cell Disease ◽  
Blood Transfusion ◽  
Iron Overload ◽  
Sickle Cell ◽  
Iron Concentration ◽  
Thalassemia Major ◽  
Iron Loading ◽  
Cell Disease ◽  
Liver Iron Concentration ◽  
Liver Iron

Abstract The aims of this review are to highlight the mechanisms and consequences of iron distribution that are most relevant to transfused sickle cell disease (SCD) patients and to address the particular challenges in the monitoring and treatment of iron overload. In contrast to many inherited anemias, in SCD, iron overload does not occur without blood transfusion. The rate of iron loading in SCD depends on the blood transfusion regime: with simple hypertransfusion regimes, rates approximate to thalassemia major, but iron loading can be minimal with automated erythrocyte apheresis. The consequences of transfusional iron overload largely reflect the distribution of storage iron. In SCD, a lower proportion of transfused iron distributes extrahepatically and occurs later than in thalassemia major, so complications of iron overload to the heart and endocrine system are less common. We discuss the mechanisms by which these differences may be mediated. Treatment with iron chelation and monitoring of transfusional iron overload in SCD aim principally at controlling liver iron, thereby reducing the risk of cirrhosis and hepatocellular carcinoma. Monitoring of liver iron concentration pretreatment and in response to chelation can be estimated using serum ferritin, but noninvasive measurement of liver iron concentration using validated and widely available MRI techniques reduces the risk of under- or overtreatment. The optimal use of chelation regimes to achieve these goals is described.

Iron Stores in Patients with Myelodysplasia and Aplastic Anemia.

Blood ◽  
2006 ◽  
Vol 108 (11) ◽  
pp. 3726-3726
Author(s):  
Peter Nielsen ◽  
Tim H. Bruemmendorf ◽  
Regine Grosse ◽  
Rainer Engelhardt ◽  
Nicolaus Kroeger ◽  
...  
Keyword(s):  
Risk Factor ◽  
Iron Overload ◽  
Aplastic Anemia ◽  
Myelodysplastic Syndromes ◽  
Iron Concentration ◽  
Dry Weight ◽  
Iron Toxicity ◽  
Intervention Threshold ◽  
Liver Iron Concentration ◽  
Liver Iron

Abstract Patients with myelodysplastic syndromes (MDS), osteomyelofibrosis (OMF), or severe aplastic anemia (SAA) suffer from ineffective erythropoiesis due to pancytopenia, which is treated with red blood cell transfusion leading to iron overload. Especially in low-risk patients with mean survival times of > 5 years, potentially toxic levels of liver iron concentration (LIC) can be reached. We hypothesize that the higher morbidity seen in transfused patients may be influenced by iron toxicity. Following a meeting in Nagasaki 2005, a consensus statement on iron overload in myelodysplastic syndromes has been published, however, there is still no common agreement about the initiation of chelation treatment in MDS patients. In the present study, a total of 67 transfused patients with MDS (n = 20, age: 17 – 75 y), OMF (n = 4, age: 48 – 68 y), SAA (n = 43, age: 5 – 64 y) were measured by SQUID biomagnetic liver susceptometry (BLS) and their liver and spleen volumes were scanned by ultrasound at the Hamburg biosusceptometer. Less than 50 % were treated with DFO. LIC (μg/g-liver wet weight, conversion factor of about 6 for μg/g-dry weight) and volume data were retrospectively analyzed in comparison to ferritin values. Additionally, 15 patients (age: 8 – 55 y) between 1 and 78 months after hematopoietic cell transplantation (HCT) were measured and analyzed. LIC values ranged from 149 to 8404 with a median value of 2705 μg/g-liver, while serum ferritin (SF) concentrations were between 500 and 10396 μg/l with a median ratio of SF/LIC = 0.9 [(μg/l)/(μg/g-liver)] (range: 0.4 to 5.2). The Spearman rank correlation between SF and LIC was found to be highly significant (RS = 0.80, p < 0.0001), however, prediction by the linear regression LIC = (0.83± 0.08)·SF was poor (R2 = 0.5) as found also in other iron overload diseases. Although iron toxicity is a long-term risk factor, progression of hepatic fibrosis has been observed for LIC > 16 mg/g dry weight or 2667 μg/g-liver (Angelucci et al. Blood2002; 100:17–21) within 60 months and significant cardiac iron levels have been observed for LIC > 350 μmol/g or 3258 μg/g-liver (Jensen et al. Blood2003; 101:4632-9). The Angelucci threshold of hepatic fibrosis progression was exceeded by 51 % of our patients, while 39 % were exceeding the Jensen threshold of potential risk of cardiac iron toxicity. The total body iron burden is even higher as more than 50 % of the patients had hepatomegaly (median liver enlargement factor 1.2 of normal). A liver iron concentration of about 3000 μg/g-liver or 18 mg/g-dry weight has to be seen as latest intervention threshold for chelation treatment as MDS patients are affected by more than one risk factor. A more secure intervention threshold would be a LIC of 1000 μg/g-liver or 4 – 6 mg/g-dry weight, corresponding with a ferritin level of 900 μg/l for transfused MDS patients. Such a LIC value is not exceeded by most subjects with heterozygous HFE-associated hemochromatosis and is well tolerated without treatment during life-time. Non-invasive liver iron quantification offers a more reliable information on the individual range of iron loading in MDS which is also important for a more rational indication for a chelation treatment in a given patient.

Comparing the Value of Serum Ferritin, Transfusion History and Magnetic Resonance Imaging for the Prediction of Iron Overload In MDS and AML Patients Undergoing Allogeneic Stem Cell Transplantation.

Blood ◽  
2010 ◽  
Vol 116 (21) ◽  
pp. 3493-3493
Author(s):  
Martin Wermke ◽  
Jan Moritz Middeke ◽  
Nona Shayegi ◽  
Verena Plodeck ◽  
Michael Laniado ◽  
...  
Keyword(s):  
Iron Overload ◽  
Serum Ferritin ◽  
Iron Content ◽  
Iron Concentration ◽  
Transferrin Saturation ◽  
Resonance Imaging ◽  
Liver Iron Concentration ◽  
Liver Iron ◽  
Liver Iron Content ◽  
Transfusion History

Abstract Abstract 3493 An increased risk for GvHD, infections and liver toxicity after transplant has been attributed to iron overload (defined by serum ferritin) of MDS and AML patients prior to allogeneic hematopoietic stem cell transplantation (allo-HSCT). Nevertheless, the reason for this observation is not very well defined. Consequently, there is a debate whether to use iron chelators in these patients prior to allo-HSCT. In fact, serum ferritin levels and transfusion history are commonly used to guide iron depletion strategies. Both parameters may inadequately reflect body iron stores in MDS and AML patients prior to allo-HSCT. Recently, quantitative magnetic resonance imaging (MRI) was introduced as a tool for direct measurement of liver iron. We therefore aimed at evaluating the accurateness of different strategies for determining iron overload in MDS and AML patients prior to allo-HSCT. Serologic parameters of iron overload (ferritin, iron, transferrin, transferrin saturation, soluble transferrin receptor) and transfusion history were obtained prospectively in MDS or AML patients prior to allo-SCT. In parallel, liver iron content was measured by MRI according to the method described by Gandon (Lancet 2004) and Rose (Eur J Haematol 2006), respectively. A total of 20 AML and 9 MDS patients (median age 59 years, range: 23–74 years) undergoing allo-HSCT have been evaluated so far. The median ferritin concentration was 2237 μg/l (range 572–6594 μg/l) and patients had received a median of 20 transfusions (range 6–127) before transplantation. Serum ferritin was not significantly correlated with transfusion burden (t = 0.207, p = 0.119) but as expected with the concentration of C-reactive protein (t = 0.385, p = 0.003). Median liver iron concentration measured by MRI was 150 μmol/g (range 40–300 μmol/g, normal: < 36 μmol/g). A weak but significant correlation was found between liver iron concentration and ferritin (t = 0.354; p = 0.008). The strength of the correlation was diminished by the influence of 5 outliers with high ferritin concentrations but rather low liver iron content (Figure 1). The same applied to transfusion history which was also only weakly associated with liver iron content (t = 0.365; p = 0.007). Levels of transferrin, transferrin saturation, total iron and soluble transferrin receptor did not predict for liver iron concentration. Our data suggest that serum ferritin or transfusion history cannot be regarded as robust surrogates for the actual iron overload in MDS or AML patients. Therefore we advocate caution when using one of these parameters as the only trigger for chelation therapy or as a risk-factor to predict outcome after allo-HSCT. Figure 1. Correlation of Liver iron content with Ferritin. Figure 1. Correlation of Liver iron content with Ferritin. Disclosures: No relevant conflicts of interest to declare.

scholarly journals Assessment of Iron Overload in the Pediatric Allogeneic HSCT Recipient

Blood ◽  
2018 ◽  
Vol 132 (Supplement 1) ◽  
pp. 5735-5735
Author(s):  
Brittany Paige DePriest ◽  
Mikey Huang
Keyword(s):  
Bone Marrow ◽  
Iron Overload ◽  
Iron Concentration ◽  
Marrow Transplant ◽  
Moderate Correlation ◽  
Liver Iron Concentration ◽  
Liver Iron ◽  
Post Transplant ◽  
Transplant Patients ◽  
T2 Mri

Abstract Background: Owing to improved strategies in pediatric bone marrow transplantation, a larger number of transplanted children are now becoming long term survivors. These post-transplant patients remain at risk for late complications including iron overload, which has the potential to impair quality of life and adversely affect later outcomes. While literature has previously focused on iron overload in the adult sickle cell patient, there has been minimal research into its effect on the pediatric bone marrow transplant recipient. Thus, no current guidelines exist for screening, management or treatment of iron overload in this patient population. Our study focuses specifically on this population and reports the relationship between number of PRBC transfusions and current diagnostic tools. Objectives: To identify the presence or absence of correlation between the number of red blood cell transfusions and indicators of iron overload via two different modalities: ferritin values and the T2* MRI liver iron concentration (Ferriscan). Methods: A retrospective chart review of the allogeneic pediatric bone marrow transplant patients over the past 5 years at a single center (n = 32). Quantitative data obtained which included number of PRBC transfusions, ferritin, and T2* MRI LIC. Correlation analysis subsequently performed between pre-and post-transplant values. Results: There was significant (p < 0.001) moderate correlation (r = 0.62) between the number of pre-transplant PRBC transfusions and the pre-transplant ferritin value. No significant (p >0.1) correlation between the number of pre-transplant PRBC transfusions and the pre-transplant T2* LIC. Also, no significant (p > 0.1) correlation between pre-transplant ferritin and T2* LIC. The total number of PRBC transfusions up to 100 days post-transplant did have significant (p = 0.008) moderate correlation (r= 0.62) with post-transplant ferritin values. There was significant (p = 0.01) strong correlation (r= 0.87) between the total number of PRBC transfusions up to 100 days post-transplant with post-transplant T2*LIC values. No significant correlation (p > 0.1) between post-transplant ferritin and T2* MRI LIC values. Conclusions: In terms of modalities utilized for evaluation of iron overload in the pediatric allogeneic BMT population, no significant correlation exists between ferritin values and T2* MRI liver iron concentration values. While ferritin is an acceptable screening tool the post-transplant T2*MRI LIC is a more accurate diagnostic indicator of transfusion burden. Future studies will be used to explore associated adverse outcomes of patients diagnosed with iron overload. Disclosures No relevant conflicts of interest to declare.

scholarly journals Estimation and quantification of liver iron concentration by magnetic resonance imaging

2019 ◽  
Vol 7 (11) ◽  
pp. 4266
Author(s):  
Vipul V. Chemburkar ◽  
Archit A. Gupta ◽  
Devdas S. Shetty ◽  
Ruchi R. Agarwal
Keyword(s):  
Iron Overload ◽  
Wide Spectrum ◽  
Iron Concentration ◽  
Dry Weight ◽  
Accurate Estimation ◽  
Liver Iron Concentration ◽  
Liver Iron ◽  
Cross Sectional ◽  
Male Predominance ◽  
Duration Of Disease

Background: Liver iron overload is considered to be the histological hallmark of genetic hemochromatosis. The accurate estimation of iron overload is important to establish the diagnosis of hemochromatosis. The aim of the present study was to estimate T2* liver value, quantify liver iron concentration (in milligram of iron per gram dry weight) and find out the appropriate therapy for patients with iron overload according to severity index.Methods: A cross-sectional observational study was carried out in Department of Radio Diagnosis, at B.Y.L. Nair Hospital and medical college, Mumbai from June 2017 to August 2018. A total of 50 cases were enrolled for the present study.Results: Male predominance (66.0%) was seen. Mean duration of disease among the studied cases was 10.52±6.06 years, with minimum and maximum duration of disease of 1 and 26 years respectively. Eight percent had compliance to visit and treatment among the enrolled cases.Conclusions: MRI was concluded to be potentially useful non-invasive method for evaluating liver iron stones in a wide spectrum of haematological and liver diseases.

scholarly journals Discrepancy between Serum Ferritin and Liver Iron Concentration in a Patient with Hereditary Hemochromatosis – The Value of T2* MRI

2020 ◽  
Vol 13 (2) ◽  
pp. 712-715
Author(s):  
Mustafa A. Al-Tikrity ◽  
Mohamed A. Yassin
Keyword(s):  
Iron Overload ◽  
Serum Ferritin ◽  
Ferritin Level ◽  
Hereditary Hemochromatosis ◽  
Iron Concentration ◽  
Hfe Gene ◽  
Liver Iron Concentration ◽  
C282y Mutation ◽  
Liver Iron ◽  
T2 Mri

Primary hemochromatosis is an inherited disorder, and the homeostatic iron regulator (HFE) gene C282Y mutation is a common cause of hemochromatosis in Europe. We are reporting a case of a 56-year-old female known to have hemochromatosis with the HFE gene C282Y mutation with a serum ferritin level of 482 μg/L who underwent heart and liver T2* MRI which showed no evidence of iron overload – neither in the heart nor in the liver. This indicates that there is a discrepancy between serum ferritin and liver iron concentration by MRI and the superiority of T2* MRI in diagnosis and follow-up of iron overload in patients with hereditary hemochromatosis.

Serum Ferritin a Predictor of Iron Overload in Patients with Thalassemia and Sickle Cell Disease?.

Blood ◽  
2004 ◽  
Vol 104 (11) ◽  
pp. 3789-3789 ◽  
Author(s):  
Zahra Pakbaz ◽  
Roland Fischer ◽  
Richard Gamino ◽  
Ellen B. Fung ◽  
Paul Harmatz ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Iron Overload ◽  
Sickle Cell ◽  
Serum Ferritin ◽  
Iron Concentration ◽  
Cell Disease ◽  
Liver Iron Concentration ◽  
Liver Iron ◽  
Iron Stores ◽  
Significant Difference

Abstract Introduction: Monitoring iron overload by serum ferritin in patients with hemosiderosis is still a routine practice although its limitations are widely studied and well known. Using non-invasive liver iron assessment by quantitative MRI or by biomagnetic liver susceptometry (BLS) with SQUID biomagnetometers would be the better alternative, however, these methods are available at only a few centers worldwide. Objective: To determine the relationship between serum ferritin (SF) and liver iron concentration (LIC), measured by BLS at CHRCO, in patients with different types of hemosiderosis. Methods and Patients: A total of 97 patients with thalassemia (TM: 3 to 52 y, 54% females) and 39 patients with sickle cell disease (SCD: 5 to 49 y, 60% female) were prospectively assessed for LIC and SF. Both tests were performed within 2 weeks of each other. Most patients with TM and SCD were chronically transfused, while 10 b-thalassemia intermedia (TI), 5 HbE/β-thalassemia (HbE), and 5 SCD patients were not on transfusion programs. LIC was measured by LTc SQUID biosusceptometer system (Ferritometer®, Model 5700, Tristan Technologies, San Diego, USA) under the standardized Hamburg-Torino-Oakland protocol. A non-parametric test (U-test) was utilized to analyze differences between SF and LIC data. Results: In chronically transfused TM and SCD patients, the median SF and LIC were very similar (Table I). In TI&HbE patients, ferritin results were disproportionately low with respect to LIC. In order to improve prediction of iron stores by SF, the SF/LIC ratio was calculated. There was a significant difference between the median ratios of the two groups of transfused and non- transfused thalassemia patients, 0.82 vs. 0.32 [μg/l]/[μg/gliver], respectively (p < 0.01). In SCD patients the ratio is significantly (p < 0.01) higher. Conclusion: Present data confirm ferritin to be a poor predictor of liver iron stores both in sickle cell disease and thalassemia. Relying only on ferritin to monitor iron overload in patients with hemosiderosis can be misleading, especially, in sickle cell disease and non-transfused thalassemia patients. Taking into account disease specific ferritin-LIC relations, could improve the prediction of iron stores. However, assessment of liver iron stores is the ultimate method to initiate and adjust chelation treatment in order to avoid progressive organ injury. Table I. Median values and ranges ( − ) of serum ferritin (SF) and liver iron concentration (LIC) in transfused (Tx) and non-transfused (non-Tx) hemosiderosis patients. Patient group n SF μg/l] LIC [mg/gliver ] SF:LIC Thalassemia Tx 82 1721 (209–8867) 3424 (364–7570) 0.82 (0.3–1.8) TI &HbE non-Tx 15 766 (52–2681) 2174 (226–5498) 0.32 (0.1–1.4) SCD Tx 34 2757 (400–9138) 1941 (518–6670) 1.2 (0.6–3.3)

Liver Iron Concentration Measurements Using MRI R2 in MDS Patients in a Deferasirox (Exjade®, ICL670) Phase II Study.

Blood ◽  
2006 ◽  
Vol 108 (11) ◽  
pp. 4846-4846 ◽  
Author(s):  
Peter L. Greenberg ◽  
Charles A. Schiffer ◽  
Charles Asa Koller ◽  
Barinder Kang ◽  
Jodie Decker ◽  
...  
Keyword(s):  
Iron Overload ◽  
Serum Ferritin ◽  
Serum Iron ◽  
Iron Concentration ◽  
Transferrin Saturation ◽  
Ongoing Study ◽  
Liver Iron Concentration ◽  
Liver Iron ◽  
Calculated Creatinine Clearance ◽  
L 14

Abstract Introduction: Approximately 60% of patients with myelodysplastic syndromes (MDS) require ongoing red blood cell transfusions, which can lead to significant iron overload and associated morbidities. Historically, many of these patients have not received iron chelation therapy due to burdensome administration of deferoxamine. Deferasirox (Exjade®, ICL670) is a once-daily, oral iron chelator recently approved for the treatment of chronic iron overload due to blood transfusions. This ongoing study is designed to evaluate the efficacy and safety of deferasirox in Low/Int-1-risk MDS patients. In addition, this is the first prospective, multicenter trial to evaluate liver iron concentration (LIC) using the MRI R2 parameter in this population. Methods: This ongoing study will enroll 30 patients at three US centers. Deferasirox will be administered at 20–30 mg/kg/day for 12 months. Iron burden is being monitored by monthly serum ferritin evaluations, and LIC by MRI R2 at baseline, 6 and 12 months. Serum iron, transferrin, transferrin saturation, labile plasma iron (LPI), and urinary hepcidin are being assessed throughout the study. In addition, serum creatinine, calculated creatinine clearance, echocardiograms and hematological status are being monitored. In this report, we are presenting the baseline data for the currently enrolled patients. Results: As of May 2006, 14 patients (9 male, 5 female; aged 55–81 years) were enrolled. All patients were Caucasian with equal distribution of Low- and Int-1-risk MDS. The mean interval from MDS diagnosis to screening was 4 years, ranging from &lt;1 to 12 years. The table summarizes baseline iron parameters in these patients: Parameter n Mean ± SD Median Range Normal range n/a, not applicable LIC, mg Fe/g dw 14 21.8 ± 11.0 23.5 3.8–40.5 &lt;1.3 Serum ferritin,μg/L 14 4645 ± 3804 3534.5 1433–15380 20–360 Serum iron, μg/dL 14 205.9 ± 26.5 200 165.9–252.0 50–160 Transferrin, mg/dL 14 143 ± 19 142.5 106–172 200–400 Transferrin saturation, % 14 113.8 ± 8.5 114 95–124 15–50 LPI, μmol/L 14 0.7 ± 0.7 0.6 0–1.9 0 Num. of lifetime transfusions 14 106.3 ± 115.5 47.5 30–352 n/a Renal function: Calculated creatinine clearance at baseline was normal (&gt;80 mL/min) in 46% of patients, mildly impaired (50–80 mL/min) in 46% and moderately impaired (30–50 mL/min) in 8% of patients. Hematological parameters: neutropenia (&lt;1800/μL): 1 patient; thrombocytopenia (&lt;100,000/μL): 3 patients; neutropenia and thrombocytopenia: 1 patient. Concurrent therapies: Revlimid: 2 patients; and hydroxyurea: 1 patient. Conclusions: Baseline iron burden in these patients demonstrates a high degree of iron overload, as measured by LIC via MRI, as well as serum ferritin, serum iron and transferrin saturation. Based on NCCN guidelines for the management of iron overload, the degree of iron overload observed meets criteria for treatment. This ongoing study is assessing the safety and efficacy of deferasirox in this population.

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