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Abstrak :
Latar Belakang: Harapan hidup pasien thalasemia bergantung transfusi bertambah baik karena transfusi darah dan terapi kelasi besi yang sesuai. Penyakit jantung akibat toksisitas besi tetap menjadi penyebab utama kematian pada pasien thalasemia bergantung transfusi. MRI T2* jantung dapat mendeteksi dini toksisitas besi di jantung dan dapat mengevaluasi hasil pengobatan dengan membandingkan nilai T2* pra dan pasca terapi kelasi besi. Tujuan Penelitian: Penelitian ini bertujuan mendapatkan profil perbaikan toksisitas besi di jantung pada pasien thalasemia dewasa bergantung transfusi. Penelitian ini juga bertujuan untuk melihat kesesuaian antara perbaikan nilai T2* jantung dengan perbaikan feritin serum dan saturasi transferin. Metode Penelitian: pre and post test dengan data sekunder retrospektif pada pasien dewasa thalasemia bergantung transfusi yang kontrol di poliklinik thalasemia Kiara dan poliklinik dewasa hematologi-onkologi medik RSUPN Cipto Mangukusumo. Penelitian dilakukan pada bulan Juli-Desember 2019. Data sekunder diperoleh dari rekam medis dan registri pasien thalasemia berupa riwayat medis, jenis obat kelasi besi, nilai T2* jantung satu tahun berturut-turut, kadar feritin serum dan saturasi transferin. Analisis data berupa data deskriptif dan uji marginal homogeneity serta uji kappa. Hasil: Sebanyak 115 pasien dilibatkan dalam penelitian ini. Terdapat perbaikan T2* jantung sebanyak 7,0% dan menetap baik (T2* jantung tetap >20 milidetik) sebanyak 72,2%. Tidak terdapat kesesuaian antara perbaikan nilai T2* jantung dengan perbaikan feritin serum (nilai kappa = 0,044) dan perbaikan nilai T2* jantung dengan saturasi transferin ( nilai kappa = 0,011). Simpulan: Perbaikan toksisitas besi di jantung pasca terapi kelasi besi sebanyak 7,0% dan menetap baik sebanyak 72,2%. Tidak terdapat kesesuaian antara perbaikan nilai T2* jantung dengan perbaikan kadar feritin serum dan saturasi transferin.

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Background: Life expectancy of the transfusion dependent thalassemia patients is getting better because of blood transfusion and appropriate iron chelation therapy. Heart disease due to iron toxicity remains the leading cause of death in thalassemia patients who need transfusion. MRI T2* can allow to detect premature iron toxicity in the heart and can evaluate the results by comparing myocardial T2* pre and post iron chelation therapy. Objectives: This study aims to obtain a profile of improvement in cardiac iron toxicity in adult thalassemia patients who need transfusion. This study also supports to see aggrement between improvement in myocardial T2* with improved serum ferritin level and transferrin saturation. Methods: pre and post test with retrospective secondary data in adult thalassemia patients requiring controlled transfusions in Kiara thalassemia clinic and hematology-medical oncology clinic Cipto Mangukusumo General Hospital. The study was conducted in July-Desember 2019. Data were obtained from medical records and thalassemia registry, which consisted of medical history, type of chelation, myocardial T2* within one year, serum ferritin level and transferrin saturation. Data analysis was performed in descriptive data and marginal homogeneity test and Kappa test. Results: A total of 115 patients were included in this study. There was an improvement of a myocardial T2* in 7.0% patients and persistently good (myocardial T2* remains >20 milliseconds) in 72.2%. There was no agreement between improvement in myocardial T2* with improvement in serum ferritin level (kappa value 0.044) and improvement in myocardial T2* with transferrin saturation (kappa value 0.011). Conclusion: Improvement of cardiac iron toxicity after iron chelation therapy was 7.0% and persistently good in 72.2%. There was no agreement between the improvement in myocardial T2* with improvement in serum ferritin level and transferrin saturation.

Abstrak :
Latar Belakang: Harapan hidup pasien thalasemia bergantung transfusi bertambah baik karena transfusi darah dan terapi kelasi besi yang sesuai. Penyakit jantung akibat toksisitas besi tetap menjadi penyebab utama kematian pada pasien thalasemia bergantung transfusi. MRI T2* jantung dapat mendeteksi dini toksisitas besi di jantung dan dapat mengevaluasi hasil pengobatan dengan membandingkan nilai T2* pra dan pasca terapi kelasi besi. Tujuan Penelitian: Penelitian ini bertujuan mendapatkan profil perbaikan toksisitas besi di jantung pada pasien thalasemia dewasa bergantung transfusi. Penelitian ini juga bertujuan untuk melihat kesesuaian antara perbaikan nilai T2* jantung dengan perbaikan feritin serum dan saturasi transferin. Metode Penelitian: pre and post test dengan data sekunder retrospektif pada pasien dewasa thalasemia bergantung transfusi yang kontrol di poliklinik thalasemia Kiara dan poliklinik dewasa hematologi-onkologi medik RSUPN Cipto Mangukusumo. Penelitian dilakukan pada bulan Juli-Desember 2019. Data sekunder diperoleh dari rekam medis dan registri pasien thalasemia berupa riwayat medis, jenis obat kelasi besi, nilai T2* jantung satu tahun berturut-turut, kadar feritin serum dan saturasi transferin. Analisis data berupa data deskriptif dan uji marginal homogeneity serta uji kappa. Hasil: Sebanyak 115 pasien dilibatkan dalam penelitian ini. Terdapat perbaikan T2* jantung sebanyak 7,0% dan menetap baik (T2* jantung tetap >20 milidetik) sebanyak 72,2%. Tidak terdapat kesesuaian antara perbaikan nilai T2* jantung dengan perbaikan feritin serum (nilai kappa = 0,044) dan perbaikan nilai T2* jantung dengan saturasi transferin ( nilai kappa = 0,011). Simpulan: Perbaikan toksisitas besi di jantung pasca terapi kelasi besi sebanyak 7,0% dan menetap baik sebanyak 72,2%. Tidak terdapat kesesuaian antara perbaikan nilai T2* jantung dengan perbaikan kadar feritin serum dan saturasi transferin.

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Background: Life expectancy of the transfusion dependent thalassemia patients is getting better because of blood transfusion and appropriate iron chelation therapy. Heart disease due to iron toxicity remains the leading cause of death in thalassemia patients who need transfusion. MRI T2* can allow to detect premature iron toxicity in the heart and can evaluate the results by comparing myocardial T2* pre and post iron chelation therapy. Objectives: This study aims to obtain a profile of improvement in cardiac iron toxicity in adult thalassemia patients who need transfusion. This study also supports to see aggrement between improvement in myocardial T2* with improved serum ferritin level and transferrin saturation. Methods: pre and post test with retrospective secondary data in adult thalassemia patients requiring controlled transfusions in Kiara thalassemia clinic and hematology-medical oncology clinic Cipto Mangukusumo General Hospital. The study was conducted in July-Desember 2019. Data were obtained from medical records and thalassemia registry, which consisted of medical history, type of chelation, myocardial T2* within one year, serum ferritin level and transferrin saturation. Data analysis was performed in descriptive data and marginal homogeneity test and Kappa test. Results: A total of 115 patients were included in this study. There was an improvement of a myocardial T2* in 7.0% patients and persistently good (myocardial T2* remains >20 milliseconds) in 72.2%. There was no agreement between improvement in myocardial T2* with improvement in serum ferritin level (kappa value 0.044) and improvement in myocardial T2* with transferrin saturation (kappa value 0.011). Conclusion: Improvement of cardiac iron toxicity after iron chelation therapy was 7.0% and persistently good in 72.2%. There was no agreement between the improvement in myocardial T2* with improvement in serum ferritin level and transferrin saturation.

Abstrak :
Latar Belakang: Transfusi darah dan kelasi besi merupakan terapi utama thalasemia khususnya pada thalassemia beta mayor yang bergantung transfusi. Harapan hidup dan prognosis pada pasien pasien tersebut telah bertambah baik dalam beberapa dekade terakhir. Namun demikian gangguan pada jantung masih merupakan penyebab mortalitas utama pada pasien thalassemia beta mayor bergantung transfusi. Toksisitas besi pada jantung merupakan penyebab utama kelainan jantung pada pasien-pasien tersebut. Modalitas utama untuk deteksi toksisitas besi pada jantung adalah dengan MRIT2 yang kesediaannya terbatas. Tujuan: Penelitian ini bertujuan mendapatkan profil toksisitas besi pada jantung penderita thalassemia beta bergantung transfusi. Penelitian ini juga bertujuan melihat korelasi antara toksisitas besi dengan fungsi sistolik jantung, fungsi diastolik jantung dan kadar NTproBNP. Selain itu penelitian ini bertujuan untuk melihat korelasi muatan besi dengan toksisitas besi pada jantung, fungsi sistolik jantung, fungsi diastolik jantung dan kadar NTproBNP. Metode: Penelitian potong lintang pada penderita dewasa dengan thalassemia beta mayor yang bergantung transfusi di Poliklinik Thalasemia Dewasa RSCM Jakarta pada bulan Desember 2017. Data sekunder diperoleh dari rekam medik pasien berupa riwayat medis, hasil lab, hasil pemerikssan MRI T2 dan ekokardiografi. Dilakukan pemeriksaan fisik dan pengambilan darah untuk pemeriksaan NTproBNP. Analisis data berupa data deskriptif dan uji korelasi dengan uji Pearson dan Spearman. Hasil: Sebanyak 62 orang pasien dilibatkan dalam studi. Median untuk T2 jantung adalah 24,96 ms. Terdapat 27,4 pasien dengan hemosiderosis jantung yang ringan-sedang dan 11,3 dengan hemosiderosis jantung yang berat. Tidak terdapat korelasi antara toksisitas besi pada jantung dengan fraksi ejeksi, ratio E/A maupun kadar NTproBNP. Terdapat korelasi lemah antara ferritin serum dan toksisitas besi pada jantung r=-0,312, p=0,007. Terdapat korelasi lemah antara ferritin serum dan fraksi ejeksi r=-0,281, p=0,013. Simpulan: Toksisitas besi pada jantung ditemukan pada 38,7 penderita dewasa dengan thalassemia beta bergantung transfusi. Tidak terdapat korelasi antara toksisitas besi pada jantung dengan fungsi sistolik, fungsi diastolik mauapun kadar NTproBNP pada penderita dewasa dengan thalssemia beta bergantung transfusi. Terdapat korelasi lemah antara ferritin serum dengan toksisitas besi pada jantung dan fungsi sitolik jantung. Tidak terdapat korelasi antara muatan besi dengan NTproBNP.

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Background Regular blood transfusions and chelation are the mainstay of treatment in TDT patients. This has improved the survival and prognosis of the patients. Cardiac complications still remain to be the main cause of mortality. Cardiac iron toxicity is the main cause of complications leading to cardiac failure. MRI T2 is the gold standard for diagnosing cardiac iron toxicity, however this facility is limited in Indonesia.Objective. This study aimed to get a profile of the cardiac iron toxicity in adult TDT beta major patients to obtain a correlation of cardiac iron toxicity with cardiac function and NTproBNP levels and to evaluate if there is any correlation between iron overload with cardiac iron toxicity, cardiac function and NTproBNP levels. Methods Cross sectional study was done which included thalassemia beta major transfusion dependent patients from the adult thalassemia policlinic of RSCM hospital during desember 2017. Data was obtained from the medical records including history, laboratory results and results of MRIT2 and echocardiography. Physical examination was done and blood drawn for NTproBNP estimation. Data was analysed for descriptive data and correlation tests using pearsons or spearman rsquo s test. Results 62 patients were included in the study. Median cardiac T2 was 24.96 ms. 27.4 patients had mild moderate cardiac hemosiderosis and 11.3 had severe cardiac siderosis. There was no correlation between cardiac iron toxicity and ejection fraction, E A ratio and NTproBNP levels. There was a weak correlation between ferritin levels and cardiac toxicity r 0,312, p 0,007 as well as with ejection fraction r 0,281, p 0,013. There was no correlation between ferritin levels and E A or NTproBNP. There was no correlation between transferrin saturation levels with cardiac iron toxicity, ejection fraction, E A ratio as well as NTproBNPConclusion Cardiac iron toxicity was seen in 38,7 of TDT patients in this study. There was no correlation between cardiac toxicity and cardiac functions as well as NTproBNP levels. There was a weak correaltion between ferritin levels with cardiac toxicity and systolic function but no correlation with diastolic function. There was no correlation between transferrin saturation levels with cardiac toxicity, systolic function, diatolic function as well NtproBNP levels.