Serum Ferritin and Cardiac/Liver Magnetic Resonance Imaging In Evaluating Iron Overload for Patients with Bone Marrow Failure Conditions Undergoing Non-Myeloablative HSCT

Blood ◽  
2010 ◽  
Vol 116 (21) ◽  
pp. 1331-1331
Author(s):  
Victoria J Tindell ◽  
Victoria T Potter ◽  
Rachel Kesse-Adu ◽  
Laura Reiff-Zall ◽  
Aloysius Y Ho ◽  
...  

Abstract Abstract 1331 Several groups have identified iron overload, in terms of raised pre-transplant serum ferritin levels, as an independent adverse prognostic factor for patients undergoing myeloablative HSCT. While serum ferritin has been used as a common marker in clinical studies to evaluate the impact of iron overload following allogeneic transplantation, there are limitations to its use with it being an acute phase reactant, as well as its lack of specificity for predicting end-organ toxicities. Patients undergoing HSCT for bone marrow failure (BMF) syndromes usually have a significant red cell transfusion history, and although the majority of these patients receive non-myeloablative HSCT regimens, it is unclear as to the impact of iron overload in these patients on subsequent transplant outcomes. In order to address these questions, we performed a prospective study evaluating the pre-transplant serum ferritin together with concurrent T2* cardiac magnetic resonance imaging (MRI) and R2 liver MRI in 18 patients with BMF syndromes undergoing allogeneic HSCT. The diagnosis of the patients included MDS (RCMD/hypoplastic MDS) =10, acquired aplastic anaemia =7, fanconi anaemia =1. The median age of the patients at transplantation was 42 years, and all patients received a T-cell depleted non-myeloablative HSCT. All patients were transfusion dependent pre-HSCT, with a median number of red cell transfusions of 45 (range: 8–115). Pre-HSCT ferritin was performed within 2 weeks of HSCT, and the results were correlated with albumin and C-reactive protein to reduce the impact of ferritin as an acute phase reactant. T2* and R2 MRI were similarly performed within 2 weeks of HSCT. The median pre-HSCT ferritin was significantly raised at 2119 ug/l(range: 559–12235). In contrast, the T2* cardiac MRI was normal for all but one patient who had evidence of mild cardiac iron overload. All patients had a corresponding cardiac echocardiogram performed with an ejection fraction within normal limits. For the liver T2* MRI, 7 patients had evidence of none or mild hepatic iron overload, while 11 patients had moderate to severe iron overload. There was no correlation between pre-HSCT transfusion burden and serum ferritin levels. Furthermore, there was no correlation between either the transfusion burden or serum ferritin, and the T2*MRI readings. In terms of HSCT outcome, the median time to neutrophil engraftment was 14 days. 2 patients had primary graft failure and only 1 patient died within 100 days due to an intra-cerebral haemorrhage. No patients had any clinical features of hepatic veno-occlusive disease (VOD), and 5 patients had evidence of grade I-II acute grade versus-host-disease. Data were also collected on the incidence of bacterial, fungal and viral infections post-HSCT for the cohort. There was however no significant association between transfusion burden, serum ferritin or T2* imaging and any of the HSCT outcomes (engraftment/day 100 TRM, GvHD, VOD or infections). In the context of heavily transfused BMF patients receiving allogeneic HSCT, serum ferritin does not correlate with end-organ deposition of iron. Despite the high transfusion burden in our cohort of patients, cardiac deposition of iron appears minimal while hepatic iron deposition is significant in a large proportion of patients. Reassuringly, a raised iron overload by either of the above mentioned parameters had no effect on HSCT outcomes. Our findings highlight the limitations of using serum ferritin as a marker of iron overload pre-HSCT. The role of active pre-HSCT chelation of BMF patients receiving non-myeloablative HSCT regimens remains unclear, and further studies are warranted. Disclosures: No relevant conflicts of interest to declare.

Blood ◽  
2015 ◽  
Vol 126 (23) ◽  
pp. 3368-3368
Author(s):  
Debmalya Bhattacharyya ◽  
Maitreyee Bhattacharyya ◽  
Saswata Chatterjee ◽  
Abhijit Chowdhury ◽  
Pramit Ghosh

Abstract Introduction: Transient Elastography (TE) of liver is a well established tool to measure liver stiffness, mainly used for assessment of hepatic fibrosis due to chronic hepatitis. Liver biopsy is the gold standard test for measurement of liver iron concentration (LIC) whereas T2* MRI is the best available non-invasive method for the same in thalassemia. We intended to use hepatic TE as an alternative cheaper tool to assess hepatic iron overload so that it can be applied to larger number of patients. Objective: To assess degree of liver stiffness by TE in patients with HbE beta thalassemia and correlate the findings with LIC calculation by T2* MRI of liver. Materials and Method: 53 patients with HbE beta thalassemia from the thalassemia clinic of Institute of Haematology and Transfusion Medicine, Medical College, Kolkata were enrolled for the study. Patients with known liver disease were excluded. Baseline data like HbE%, mutations, transfusion requirement, growth status, serum ferritin level etc were collected. All of them underwent TE of liver in the School of Digestive and Liver Diseases, IPGMER using the FibroScan Touch 502 machine (Di Marco et al, British Journal of Haematology, Volume 148,3, 476-479, February 2010). 20 randomly selected patients were also assessed by T2*MRI of liver for hepatic iron assessment at the same time. LIC calculation was done from T2* value (J S Hankins et al, Blood, 14 May 2009, Volume 113:20). Data were analyzed by SPSS software-19, IBM. Results: The patients with HbE beta thalassemia had a mean HbE level of 53.66 (±18.45) %. Common beta mutations [mostly IVS-1-5(G-C)] usually found in this part of India, were detected. Mean and median age of the study population was 24.11±13.11 years and 20 years, respectively. Median age of 1st transfusion was 11 years. 35.84% patients were non-transfusion dependent. 39/53 patients had facial deformity and growth retardation. Mean baseline hemoglobin was 7.10±0.76 gm/ dl. Mean serum ferritin level was 3183.66±338.45 ng/ml. TE showed 30.18 % patients had severe liver stiffness (Liver stiffness measurement, LSM >15 kPa) whereas 43.34% had minimum stiffness (LSM≤7 kPa). No significant statistical correlation was found between serum ferritin and LSM. 12/20 patients showed very high calculated LIC (>15 mg/g) and lower T2* value (<1.8 ms) whereas only 10% of them showed mildly elevated calculated LIC. Rest had intermediate LIC. Discussion: There is lack of data regarding hepatic iron overload in HbE beta thalassemia and so also from this part of India. There was a trend that higher the age, higher was the LSM irrespective of the serum ferritin level though not found statistically significant (Figure 1). Serum ferritin level was also not significantly correlated with the calculated LIC in those 20 patients assessed with T2* MRI. 2 patients with mildly elevated LIC had a high ferritin level. Preliminary report indicates that with increase in LSM there was increase in calculated LIC also. Statistical analysis revealed patients with LSM≥7.2 kPa had moderate or severe hepatic iron overload and thus undermine the need for routine T2*MRI. The cut off value signifies that patients with LSM<7.2 kPa might or might not have significantly high liver iron overload, so obviously to be assessed by T2*MRI (Table 1). Therefore use of TE may be an alternative preliminary diagnostic method to gauge hepatic iron overload in HbE beta thalassemia patients. It would be of more value in countries like India where T2* MRI facility is not yet feasible in many centers catering to huge number of HbE-beta thalassemia patients. However, further exploration with larger number of patients is necessary to establish association of LIC and LSM in a more robust way. Conclusion: In resource-poor countries like India, TE may be a relatively cheap tool to be used as a marker of hepatic iron overload in future. Table 1. Finding Cut off: ROC (TE-value and LIC categories), n=20 Positive if Greater Than or Equal Toa Sensitivity 1 - Specificity 2.3 1.00 1.00 3.4 1.00 .50 4.4 .94 .50 5.7 .88 .50 6.2 .83 .50 6.5 .77 .50 7.2 .77 .00 8.2 .72 .00 8.85 .66 .00 9.45 .61 .00 10.2 .55 .00 11.85 .50 .00 13.85 .44 .00 15.75 .38 .00 18.3 .33 .00 22.9 .27 .00 27.9 .22 .00 35.9 .16 .00 44.7 .11 .00 48.0 .05 .00 49.8 .00 .00 Table 2. The smallest cutoff value is the minimum observed test value minus 1, and the largest cutoff value is the maximum observed test value plus 1. LSM more than 7.2 had a sensitivity of 77.2 % and specificity of 100%. Figure 2. Figure 2. Disclosures No relevant conflicts of interest to declare.


Blood ◽  
2011 ◽  
Vol 118 (21) ◽  
pp. 5304-5304
Author(s):  
Surekha Tony ◽  
Shahina Daar ◽  
Shoaib Al Zadjali ◽  
Murtadha K. Al-Khabori ◽  
Mohammed El Shinawy ◽  
...  

Abstract Abstract 5304 Background: Non-transfused patients with thalassemia intermedia (TI) accumulate iron in their body due to increased gastrointestinal absorption of iron and release of iron from the macrophages. Earlier studies have revealed that serum ferritin does not reflect the severity of iron overload in non-transfused TI patients. The current study aims at evaluating the iron overload status in a group of young hypertransfused TI children. Materials and Methods: Eleven patients (mean age 13.18±4.094 years) with TI on regular follow-up at the Pediatric Thalassemia Day Care Centre, Sultan Qaboos University Hospital, Oman were included in the study after approval by the Medical Research and Ethics Committee. All patients had beta gene mutational analysis. They were diagnosed as intermedia because of their definitive TI mutation, late age at presentation (>5 years) and transfusion independence (mean baseline Hb 6.9 g/dl). Patients were treated conventionally with hypertransfusion, and chelation, as guided by their serum ferritin levels. Serum ferritin (2 monthly) was analyzed using the Beckman Coulter Access 2 Immunoassay System. Based on serum ferritin levels, patients were classified into 2 groups, group 1(six patients) and 2 (five patients) with serum ferritin levels below and above 1000 ng/ml respectively. All patients underwent cardiac T2* MRI assessment. Based on local reference values for T2*MRI, quantification of cardiac iron deposition was categorized as normal, mild, moderate and severe iron overload at values > 20 ms, 14–20 ms, 10–14 ms and < 10 ms respectively. Simultaneous liver iron T2* values were categorized into normal, mild, moderate and severe iron overload at values > 9.1 ms, 7.1–9.0 ms, 3.1– 7.0 ms and <3.0 ms respectively. Results: Patients in group 1 and 2 had mean serum ferritin levels of 817.300±244.690 ng/ml and 1983.80±662.862 ng/ml respectively (p = 0.003). Despite this very high variation in serum ferritin values, T2* MRI showed comparable hepatic iron overload status in both the groups with mean hepatic T2* value of 2.51±0.46 ms and 3.4±1.63 ms in group 1 and group 2 respectively. The difference in hepatic T2* between the 2 groups is −0.88 (95% confidence interval −2.44 to 0.68) which is statistically insignificant (p =0.23, t-test). None of the studied patients had myocardial iron deposition (overall mean 36.86±7.8 ms). Other confounders like initial ages at presentation, pre-transfusion hemoglobin levels, durations of transfusion and chelation therapies were statistically insignificant for the 2 groups. No specific pattern of beta gene sequence was noted in either group. Conclusions: We conclude in our patients with TI on hypertransfusion, serum ferritin does not reflect their moderate to severe hepatic iron overload status. Inspite of steady serum ferritin trends, evaluation of iron overload by T2* MRI and optimal chelation is strongly recommended in hypertransfused TI patients. Disclosures: No relevant conflicts of interest to declare.


Blood ◽  
2011 ◽  
Vol 118 (21) ◽  
pp. 3827-3827
Author(s):  
Fabio P S Santos ◽  
Claudia Bley ◽  
Ricardo Helman ◽  
Guilherme Fleury Perini ◽  
Leandro de Padua Silva ◽  
...  

Abstract Abstract 3827 Introduction: Transfusion dependent anemia and iron overload (IO) are associated with reduced survival in MDS. Serum ferritin is the most common method of assessing body iron content, but is also an inflammatory marker, and may not correlate with IO in specific organs. T2* magnetic resonance imaging (MRI) is a non-invasive method for detecting IO. The prevalence of IO in patients with MDS, as detected by T2* MRI, is currently unknown. Purpose: We designed a single center trial to evaluate the efficacy of T2* MRI in detection of IO in patients with MDS, determine the prevalence of iron overload in this disease and correlate MRI findings with iron indexes (ferritin, transferrin and labile plasma iron [LPI]). Material and methods: Patients with WHO-2008 defined MDS or chronic myelomonocytic leukemia (CMML), independent of transfusion requirements, were eligible. Patients receiving iron chelation therapy were excluded. Iron indexes were measured at the time of T2* MRI evaluation. Hepatic iron overload (HIO) was considered in patients with a hepatic iron concentration (HIC) ≥ 2 g/mg. Cardiac iron overload (CIO) was considered in patients with a T2* value < 20 milliseconds. Results: A total of 58 patients with MDS and two patients with CMML have been recruited. Three patients were not evaluated by MRI due to claustrophobia, so 57 patients remain for the analysis. Median age was 66 years (range 18–89). MDS subtypes by WHO included: refractory anemia (N=5), refractory anemia with ring sideroblasts (RARS; N=7), 5q- syndrome (N=4), refractory cytopenias with multilineage dysplasia (N=21), refractory anemia with excess blasts-I (N=7) and –II (N=7), unclassifiable MDS (N=3), CMML (N=2) and therapy-related MDS (N=1). Clinical features at time of MRI are presented in table 1. Median cardiac T2* value was 42 ms (range 19.7–70.1 ms), and only one patient had a T2* value indicative of CIO. Median HIC value was 3.9 g/mg (range 0.9–16 g/mg), and 68% of patients had HIO. Patients with HIO had higher ferritin levels (1182 ng/mL vs. 185 ng/mL, p<0.0001) and transferrin saturation (76% vs. 34%, p<0.0001), but no difference in LPI (0.13 vs. 0.12 mM, p=0.49). HIO was found in 76% of transfusion dependent patients, but in also 59% of patients without history of transfusions. Among patients with HIO but without a history of transfusions, 50% had RARS. Considering MRI as a gold standard for detecting HIO, a ferritin value ≥ 1.000 ng/ml had a positive predictive value (PPV) for HIO of 98% and a NPV of 48%; for transferrin saturation ≥ 50%, PPV was 91% and NPV was 55%. Conclusions: This is one of the largest studies evaluating IO in MDS by T2* MRI. The prevalence of iron overload in MDS is underestimated by using conventional iron indexes and T2* MRI can help in the early detection of IO. In some subtypes of MDS, such as RARS, mechanisms other than transfusion dependency lead to IO. Disclosures: Santos: Janssen-Cilag: Consultancy, Speakers Bureau; United Medical: Consultancy, Speakers Bureau; Novartis: Research Funding. Hamerschlak:Novartis: Research Funding.


2021 ◽  
Vol 8 (8) ◽  
pp. 1374
Author(s):  
Shailaja V. Mane ◽  
Sharad Agarkhedkar ◽  
Dyaneshwar Upase ◽  
Tushar Kalekar ◽  
P. Sindhura

Background: Frequent blood transfusions in thalassemia major is associated with iron overload in these patients. To reduce the mortality and morbidity, proper usage of iron chelators is necessary to treat iron overload. Cardiac magnetic resonance imaging (MRI) guides in quantification of iron overload in heart. The purpose of this study is to see the correlation between serum ferritin level and T2* MRI in patients with beta thalassemia major.Methods: Period of the study is September 2018 to September 2020. Total 25 patients diagnosed with β-thalassemia major above 5 years of age were enrolled in the study. They were on regular transfusions. Cardiac T2* MRI was done in these patients and correlated with serum ferritin levels.Results: There was no significant correlation observed between cardiac T2* MRI and serum ferritin values (p=0.66, r=-0.094).Conclusions: Our results showed no significant correlation between serum ferritin and cardiac T2* MRI values. Ferritin alone cannot be used as index of myocardial iron overload in thalassemia major.


Author(s):  
Ashraf M. El Sherif ◽  
Ahmed S. Ibrahim ◽  
Mohamed A. Elsayed ◽  
Ahmed S. Abdelhakim ◽  
Ahlam M. Ismail

Abstract Background Thalassemia is the most prevalent single-gene disorder. Myocardial and hepatic iron depositions lead to complications and eventually death. We aimed to assess the diagnostic efficacy of magnetic resonance imaging T2* (MRI T2*) in quantifying iron overload in liver and heart in transfusion-dependent B-thalassemia major (TDT) children. Methods Prospective clinical study was carried on sixty children diagnosed with TDT. All of them underwent laboratory investigations, including CBC, serum iron, and ferritin levels. MRI T2* of the heart and liver was carried out to measure the iron overload and estimate the left ventricular ejection fraction (LVEF). Results Thirty-eight males and 22 females with TDT with a mean age of 13.23 years were included. Twenty cases (33.3%) had severe liver iron overload, while 36 (60%) had normal cardiac iron. There was a moderate significant negative association between hepatic and cardiac iron deposition (P = 0.03). All cases with severe cardiac iron overload had impaired LVEF below 56%. A non-significant positive association was noticed between cardiac iron deposition and LVEF in T2* (P = 0.08). A moderate negative significant association was detected between hepatic iron deposition and serum ferritin, while a fair negative significant association was found between serum ferritin and cardiac iron deposition with P values of 0.04 and 0.02, respectively. Conclusion MRI T2* is the gold standard for monitoring and follow-up of iron overload in the heart and liver. It should be routinely performed in all TDT children as liver iron, and serum ferritin do not reflect cardiac iron overload.


Blood ◽  
2016 ◽  
Vol 128 (22) ◽  
pp. 4829-4829 ◽  
Author(s):  
Mehran Karimi ◽  
Fatemeh Amirmoezi ◽  
Sezaneh Haghpanah ◽  
Seyed pouria Ostad ◽  
Mehrzad Lotfi ◽  
...  

Abstract Background: B-Thalassemia intermediate (B-TI) is a genetic disease that is milder than beta thalassemia major. The accumulation of iron in different organs causes tissue damage. The T2* magnetic resonance imaging (MRI) technique is currently the gold standard for iron load detection. However, it is expensive and needs an expert radiologist to report findings. Therefore, we conducted this study to determine an optimal cut-off value of ferritin in proportion to T2 MRI for early detection of cardiac and hepatic iron overload in patients with beta thalassemia intermediate. Methods: This cross-sectional study was conducted on 108 patients with B-TI who referred to tertiary Hospital, Shiraz University of Medical Sciences, Shiraz, Iran. Serum ferritin, hepatic and cardiac T2 MRI were assessed. The ROC curve was used to determine the sensitivity and specificity of cut-off value. Results: Serum ferritin levels showed a statistically significant negative correlation with T2 hepatic MRI (r= -0.290, P value=0.003) and positive correlation with LIC (r= 0.426, P value ˂ 0.001) in the patients with BTI. However, T2 cardiac MRI was not significantly correlated with serum ferritin levels (P value= 0.073).According to the analysis of ROC curves, the best cut-off value for ferritin to show early diagnosis of liver iron overload was 412 ng/ml. calculated sensitivities and specificities were 0.78 and 0.82 for T2 liver MRI and 0.76 and 0.86 for liver iron concentration (LIC) respectively. Conclusion: Serum ferritin levels of 412 ng/ml might be considered as a cut-off point to evaluate hepatic iron overload before using expensive, not readily available T2 MRI. This level of serum ferritin (around 500 ng/ml) could be considered for starting iron chelation therapy in patients with B-TI in areas where T2 MRI is not available. Disclosures No relevant conflicts of interest to declare.


1994 ◽  
Vol 67 (796) ◽  
pp. 339-341 ◽  
Author(s):  
S Bondestam ◽  
A Lamminen ◽  
V-J Anttila ◽  
T Ruutu ◽  
P Ruutu

Hematology ◽  
2017 ◽  
pp. 1-7 ◽  
Author(s):  
Pustika Amalia Wahidiyat ◽  
Felix Liauw ◽  
Damayanti Sekarsari ◽  
Siti Ayu Putriasih ◽  
Vasili Berdoukas ◽  
...  

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