Is serum ferritin level or T2-sequence magnetic resonance imaging more effective in predicting liver iron in transfusion-dependent thalassemia cases?
Öz
Aim: Iron overload in transfusion-dependent thalassemia patients is a condition that requires continuous chelation therapy and monitoring. Determination of serum ferritin level is considered a simple method to monitor body iron load; however, it highlights that other methods of liver iron level determination, such as magnetic resonance imaging (MRI), are more precise.
Materials and Methods: In order to contribute to understanding of liver iron load in thalassemia, liver iron level results of 14 transfusion-dependent thalassemia patients who underwent liver biopsy in preparation for stem cell transfusion were compared with liver T2 MRI and serum ferritin results.
Results: The mean serum Ferritin value was 2488.43±1520.18 mg/L. When liver iron load was evaluated according to T2*MRI results, mild iron accumulation was found in eight patients, moderate level in five patients, and advanced iron accumulation in a patient. According to the modified Scheuer classification, iron level in biopsies was grade 1 in two patients; grade 2 in seven patients; It was grade 3 in three patients and grade 4 in two patients. As the ferritin level increased, the liver iron biopsy score also increased statistically significantly (r=0.544 and p=0.044). There was a statistically significant and inverse correlation between liver T2*MRI level and liver iron biopsy score (r=-0.724 and p=0.003). Ferritin level was not found statistically significant in differentiating iron level according to liver biopsy iron score (p=0.096). The area under the ROC curve for T2*MRI measurements was statistically significant (AUC=0.967; 95% CI: 0.880-1,000 and p=0.005).
Conclusıon: In our study, we found that serum ferritin and T2 MRI results were correlated with liver biopsy iron levels. However, we found that the sensitivity and specificity of ferritin level in liver biopsy to show iron level was low, and the sensitivity and specificity of T2 MRI was high.
Anahtar Kelimeler
Thalassemia major liver iron level serum ferritin T2 magnetic resonance imaging
Kaynakça
- Telfer P, Coen PG, Christou S, Hadjigavriel M, Kolnakou A, Pangalou E, Pavlides N, Psiloines M, Simamonian K, Skordos G, Sitarou M, Angastiniotis M. Survival of medically treated thalassemia patients in Cyprus. Trends and risk factors over the period 1980-2004. Haematologica. 2006; 91: 1187-92.
- Modell B, Khan M, Darlison M, Westwood MA, Ingram D, Pennell DJ. Improved survival of thalassaemia major in the UK and relation to T2* cardiovascular magnetic resonance. J Cardiovasc Magn Reson. 2008;10:42.
- Voskaridou E, Ladis V, Kattamis A, et al. A national registry of haemoglobinopathies in Greece: deducted demographics, trends in mortality and affected births. Ann Hematol 2012; 91: 1451–8.
- Aydinok Y, Porter JB, Piga A, Elalfy M, El-Beshlawy A, Kilinç Y, Viprakasit V, Yesilipek A, Habr D, Quebe-Fehling E, Pennell DJ. Prevalence and distribution of iron overload in patients with transfusion-dependent anemias differs across geographic regions: results from the CORDELIA study. Eur J Haematol. 2015 Sep; 95 (3): 244-53.
- Mazza P, Giua R, De Marco S, Bonetti MG, Amurri B, Masi C, Lazzari G, Rizzo C, Cervellera M, Peluso A, et al. Iron overload in thalassemia: comparative analysis of magnetic resonance imaging, serum ferritin and iron content of the liver. Haematologica. 1995 Sep-Oct; 80 (5): 398-404. PMID: 8566878.
- Angelucci E, Barosi G, Camaschella C, Cappellini MD, Cazzola M, Galanello R, Marchetti M, Piga A, Tura S. Italian Society of Hematology practice guidelines for the management of iron overload in thalassemia major and related disorders. Haematologica. 2008; 93: 741- 52.
- Paschos P, Vlachaki E, Pasvanti C, Sinakos E, Kalpaka A, Klonizakis P, Perifanis V.. Safety and efficacy of combination therapy with pegylated interferon alpha-2a and ribavirin in treating patients with chronic hepatitis C and beta-thalassaemia major: a Greek single-center experience. Acta Haematol. 2011; 126: 231-3.
- Strocchio L, Locatelli F. Hematopoietic Stem Cell Transplantation in Thalassemia. Hematol Oncol Clin North Am. 2018 Apr; 32 (2): 317-28.
- Lucarelli G, Galimberti M, Giardini C, et al. Bone marrow transplantation in thal- assemia. The experience of Pesaro. Ann New York Acad Sci 1998;850:270–5.
- Turlin B, Deugnier Y. Evaluation and interpretation of iron in the liver. Semin Diagn Pathol. 1998 Nov; 15 (4): 237-45. PMID: 9845425.
- Ishak K, Baptista A, Bianchi L, Callea F, De Groote J, Gudat F, Denk H, Desmet V, Korb G, MacSween RN, et al. Histological grading and staging of chronic hepatitis. J Hepatol. 1995 Jun; 22 (6): 696-9.
- Dessì C, Leoni G, Moi P, Danjou F, Follesa I, Foschini ML, Morittu M, Zappu A, Defraia E, Bina P, Cunico A, Civolani A, Podda RA, Origa R. Thalassemia major between liver and heart: Where we are now. Blood Cells Mol Dis. 2015 Jun; 55 (1): 82-8.
- Pennell DJ, Udelson JE, Arai AE, Bozkurt B, Cohen AR, Galanello R, Hoffman TM, Kiernan MS, Lerakis S, Piga A, Porter JB, Walker JM, Wood J; American Heart Association Committee on Heart Failure and Transplantation of the Council on Clinical Cardiology and Council on Cardiovascular Radiology and Imaging. Cardiovascular function and treatment in β-thalassemia major: a consensus statement from the American Heart Association. Circulation. 2013; 128: 281-308.
- Capellini MD, Cohen A, Porter J, Taher A, Viprakasit V. Guidelines for the management of transfusion dependent Thalassaemia, Published by Thalasasemia International Federation, 2014.
- Brittenham GM. The red cell cycle. In: Brock JH, Halliday JW, Pippard MJ, Powell LW, editors. Iron Metabolism in Health and Disease. London: Saunders; 1994.
- Kolnagou A, Natsiopoulos K, Kleanthous M, Ioannou A, Kontoghiorghes GJ. Liver iron and serum ferritin levels are misleading for estimating cardiac, pancreas, splenic and total body iron load in thalassemia patients: factors influencing the heterogenic distribution of excess storage iron in organs as identified by MRI T2*. Toxicol Mech Methods. 2013; 23: 48–56.
- Eghbali A, Taherhamadi H, Shahbazi M, Bagheri B, Ebrahimi L. Association between serum ferritin level, cardiac and hepatic T2-star MRI in patients with major b-thalassemia. Iran J Pediatr Hematol Oncol. 2014; 4: 17–21.
- Perifanis V, Christoforides A, Vlachaki E, Tsatra I, Spanos G, Athanassiou-Metaxa M. Comparison of effects of different long-term iron-chelation regimens on myocardial and hepatic iron concentrations assessed with T2* magnetic resonance imaging in patients with b-thalassemia major. Int J Hematol. 2007; 86: 385–9.
- Kolnagou A, Natsiopoulos K, Kleanthous M, Ioannou A, Kontoghiorghes GJ. Liver iron and serum ferritin levels are misleading for estimating cardiac, pancreas, splenic and total body iron load in thalassemia patients: factors influencing the heterogenic distribution of excess storage iron in organs as identified by MRI T2*. Toxicol Mech Methods. 2013; 23: 48–56.
- Chuansumrit A, Laothamathat J, Sirachainan N, Sungkarat W, Wongwerawattanakoon P, Kumkrua P. Correlation between liver iron concentration determined by magnetic resonance imaging and serum ferritin in adolescents with thalassaemia disease. Paediatr Int Child Health. 2016 Aug; 36 (3): 203-8.
Transfüzyon bağımlı talasemi olgularında karaciğer demirini öngörmede serum ferritin düzeyi mi, T2 sekanslı manyetik rezonans görüntüleme mi daha etkin?
Öz
Amaç: Transfüzyon bağımlı talasemi hastalarında aşırı demir yüklenmesi, sürekli şelasyon tedavisi ve demir düzeyi izlemini gerektiren bir durumdur. Serum ferritin düzeyinin tayini, demir yükünü takip etmek için basit bir yöntem olarak kabul edilmektedir. Bununla birlikte, karaciğer demir düzeyi tayininde manyetik rezonans görüntüleme (MRG) gibi yöntemlerin daha kesin olduğunu vurgulamaktadır.
Gereç ve Yöntem: Talasemide demir yükünün anlaşılmasına katkıda bulunmak amacıyla, kök hücre transfüzyonuna hazırlık amacıyla karaciğer biyopsisi yapılan 14 transfüzyon bağımlı talasemi hastasının karaciğer demir düzeyi sonuçlarını karaciğer T2 MRG ve serum ferritin sonuçları ile karşılaştırıldı.
Bulgular: Serum ferritin değeri ortalaması 2488,43±1520,18 mg/L idi. T2*MRG sonuçlarına göre karaciğer demir yükü değerlendirildiğinde sekiz hastada hafif düzey, beş hastada orta düzey, bir hastada ileri düzey demir birikimi saptandı. Modifiye Scheuer sınıflamasına göre biyopsilerde demir düzeyi iki hastada derece 1; yedi hastada derece 2; üç hastada derece 3 ve iki hastada derece 4 olarak değerlendirildi. Ferritin düzeyi arttıkça karaciğer demir biyopsi skoru da istatistiksel anlamlı olarak artmaktaydı (r=0,544 ve p=0,044). Karaciğer T2 MRG düzeyi ile karaciğer demir biyopsi skoru arasında ise istatistiksel olarak anlamlı ve ters yönlü korelasyon mevcuttu (r=-0,724 ve p=0,003). Karaciğer biyopsi demir skoruna göre demir düzeyini ayırt etmede ferritin düzeyi istatistiksel olarak anlamlı bulunmazken (p=0,096); T2*MRG ölçümlerine ilişkin ROC eğrisi altında kalan alan istatistiksel olarak anlamlı bulundu (AUC=0,967; 95% CI: 0,880-1,000 ve p=0,005).
Sonuç: Çalışmamızda serum ferritin ve T2 MRG sonuçlarının karaciğer biyopsisi demir düzeyi ile korele olduğunu saptadık. Ancak ferritin düzeyinin karaciğer biyopsisi demir düzeyini göstermede sensitivite ve spesifitesinin düşük olduğu, T2 MRG ise sensitivite ve spesifitesinin yüksek olduğunu saptadık.
Anahtar Kelimeler
Talasemi major karaciğer demir düzeyi serum ferritin T2 manyetik rezonans görüntüleme
Kaynakça
- Telfer P, Coen PG, Christou S, Hadjigavriel M, Kolnakou A, Pangalou E, Pavlides N, Psiloines M, Simamonian K, Skordos G, Sitarou M, Angastiniotis M. Survival of medically treated thalassemia patients in Cyprus. Trends and risk factors over the period 1980-2004. Haematologica. 2006; 91: 1187-92.
- Modell B, Khan M, Darlison M, Westwood MA, Ingram D, Pennell DJ. Improved survival of thalassaemia major in the UK and relation to T2* cardiovascular magnetic resonance. J Cardiovasc Magn Reson. 2008;10:42.
- Voskaridou E, Ladis V, Kattamis A, et al. A national registry of haemoglobinopathies in Greece: deducted demographics, trends in mortality and affected births. Ann Hematol 2012; 91: 1451–8.
- Aydinok Y, Porter JB, Piga A, Elalfy M, El-Beshlawy A, Kilinç Y, Viprakasit V, Yesilipek A, Habr D, Quebe-Fehling E, Pennell DJ. Prevalence and distribution of iron overload in patients with transfusion-dependent anemias differs across geographic regions: results from the CORDELIA study. Eur J Haematol. 2015 Sep; 95 (3): 244-53.
- Mazza P, Giua R, De Marco S, Bonetti MG, Amurri B, Masi C, Lazzari G, Rizzo C, Cervellera M, Peluso A, et al. Iron overload in thalassemia: comparative analysis of magnetic resonance imaging, serum ferritin and iron content of the liver. Haematologica. 1995 Sep-Oct; 80 (5): 398-404. PMID: 8566878.
- Angelucci E, Barosi G, Camaschella C, Cappellini MD, Cazzola M, Galanello R, Marchetti M, Piga A, Tura S. Italian Society of Hematology practice guidelines for the management of iron overload in thalassemia major and related disorders. Haematologica. 2008; 93: 741- 52.
- Paschos P, Vlachaki E, Pasvanti C, Sinakos E, Kalpaka A, Klonizakis P, Perifanis V.. Safety and efficacy of combination therapy with pegylated interferon alpha-2a and ribavirin in treating patients with chronic hepatitis C and beta-thalassaemia major: a Greek single-center experience. Acta Haematol. 2011; 126: 231-3.
- Strocchio L, Locatelli F. Hematopoietic Stem Cell Transplantation in Thalassemia. Hematol Oncol Clin North Am. 2018 Apr; 32 (2): 317-28.
- Lucarelli G, Galimberti M, Giardini C, et al. Bone marrow transplantation in thal- assemia. The experience of Pesaro. Ann New York Acad Sci 1998;850:270–5.
- Turlin B, Deugnier Y. Evaluation and interpretation of iron in the liver. Semin Diagn Pathol. 1998 Nov; 15 (4): 237-45. PMID: 9845425.
- Ishak K, Baptista A, Bianchi L, Callea F, De Groote J, Gudat F, Denk H, Desmet V, Korb G, MacSween RN, et al. Histological grading and staging of chronic hepatitis. J Hepatol. 1995 Jun; 22 (6): 696-9.
- Dessì C, Leoni G, Moi P, Danjou F, Follesa I, Foschini ML, Morittu M, Zappu A, Defraia E, Bina P, Cunico A, Civolani A, Podda RA, Origa R. Thalassemia major between liver and heart: Where we are now. Blood Cells Mol Dis. 2015 Jun; 55 (1): 82-8.
- Pennell DJ, Udelson JE, Arai AE, Bozkurt B, Cohen AR, Galanello R, Hoffman TM, Kiernan MS, Lerakis S, Piga A, Porter JB, Walker JM, Wood J; American Heart Association Committee on Heart Failure and Transplantation of the Council on Clinical Cardiology and Council on Cardiovascular Radiology and Imaging. Cardiovascular function and treatment in β-thalassemia major: a consensus statement from the American Heart Association. Circulation. 2013; 128: 281-308.
- Capellini MD, Cohen A, Porter J, Taher A, Viprakasit V. Guidelines for the management of transfusion dependent Thalassaemia, Published by Thalasasemia International Federation, 2014.
- Brittenham GM. The red cell cycle. In: Brock JH, Halliday JW, Pippard MJ, Powell LW, editors. Iron Metabolism in Health and Disease. London: Saunders; 1994.
- Kolnagou A, Natsiopoulos K, Kleanthous M, Ioannou A, Kontoghiorghes GJ. Liver iron and serum ferritin levels are misleading for estimating cardiac, pancreas, splenic and total body iron load in thalassemia patients: factors influencing the heterogenic distribution of excess storage iron in organs as identified by MRI T2*. Toxicol Mech Methods. 2013; 23: 48–56.
- Eghbali A, Taherhamadi H, Shahbazi M, Bagheri B, Ebrahimi L. Association between serum ferritin level, cardiac and hepatic T2-star MRI in patients with major b-thalassemia. Iran J Pediatr Hematol Oncol. 2014; 4: 17–21.
- Perifanis V, Christoforides A, Vlachaki E, Tsatra I, Spanos G, Athanassiou-Metaxa M. Comparison of effects of different long-term iron-chelation regimens on myocardial and hepatic iron concentrations assessed with T2* magnetic resonance imaging in patients with b-thalassemia major. Int J Hematol. 2007; 86: 385–9.
- Kolnagou A, Natsiopoulos K, Kleanthous M, Ioannou A, Kontoghiorghes GJ. Liver iron and serum ferritin levels are misleading for estimating cardiac, pancreas, splenic and total body iron load in thalassemia patients: factors influencing the heterogenic distribution of excess storage iron in organs as identified by MRI T2*. Toxicol Mech Methods. 2013; 23: 48–56.
- Chuansumrit A, Laothamathat J, Sirachainan N, Sungkarat W, Wongwerawattanakoon P, Kumkrua P. Correlation between liver iron concentration determined by magnetic resonance imaging and serum ferritin in adolescents with thalassaemia disease. Paediatr Int Child Health. 2016 Aug; 36 (3): 203-8.
Ayrıntılar
| Birincil Dil | Türkçe |
|---|---|
| Konular | Sağlık Kurumları Yönetimi |
| Bölüm | Araştırma Makaleleri |
| Yazarlar | |
| Yayımlanma Tarihi | 12 Eylül 2022 |
| Gönderilme Tarihi | 11 Kasım 2021 |
| Yayımlandığı Sayı | Yıl 2022Cilt: 61 Sayı: 3 |
