Tesis

Korelasi Antara Muatan Besi Berlebih Darah dan Hati dengan Elastografi Hati pada Pasien Thalasemia Intermedia Dewasa yang Mendapatkan Transfusi Darah = Correlation of Serum Ferritin and Liver Iron Concentration with Transient Liver Elastography in Adult Thalassemia Intermedia Patient with Blood Transfusion.

Latar Belakang: Muatan besi berlebih akibat transfusi darah dan peningkatan serapan besi di saluran cerna, masih merupakan isu penting pada Thalasemia Intermedia (TI), dan dikaitkan dengan berbagai komplikasi yang meningkatkan morbiditas dan mortalitas. Pemeriksaan MRI T2* hati sebagai pemeriksaaan yang tervalidasi dalam menilai Liver Iron Concentration (LIC), merupakan pemeriksaan yang mahal dan tidak tersedia secara merata di Indonesia. Tujuan: Mengetahui gambaran muatan besi berlebih darah dan hati pada pasien thalasemia intermedia dewasa dan korelasi antara saturasi transferin, feritin serum, MRI T2* hati, dan LIC yang dinilai dengan pemeriksaan MRI T2* hati dengan nilai elastografi hati. Metode: Penelitian potong lintang pada pasien thalasemia intermedia dewasa dengan transfusi darah dan dengan atau tanpa kelasi besi di RSUPNCM dr. Cipto Mangunkusumo mulai dari bulan Agustus sampai dengan Oktober 2016, dengan total subyek penelitian sebanyak 45 orang. Dilakukan pemeriksaan saturasi transferin, feritin serum, elastografi hati, dan MRI T2* hati. Analisis statistik menggunakan uji korelasi Pearson dan Spearman. Hasil: Sebanyak 64,4% subyek penelitian adalah Thalasemia β-Hb E, dengan median (RIK) umur 33 (22) tahun. Sebanyak 84,4% subyek penelitian mendapatkan transfusi darah secara reguler. Seluruh subyek penelitian mengalami komplikasi hemosiderosis hati melalui pemeriksaan MRI T2* hati. Sebanyak 48,9% mengalami hemosiderosis hati berat, dengan nilai median MRI T2* hati 1,6 ms. rerata feritin serum adalah 2831 (1828) ng/mL, dengan nilai median saturasi transferin 66%. Nilai rerata LIC melalui pemeriksaan MRI T2* adalah 15,36 ± 7,37 mg besi/gr berat kering hati dan nilai rerata elastografi hati adalah 7,7 ± 3,8 Kpa. Uji korelasi didaptakan terdapat korelasi nilai elastografi hati dan rerata feritin serum (r = 0,651; p = 0,000), nilai elastografi hati dan LIC (r = 0,433; p = 0,003) dan korelasi negatif nilai elastografi hati dan MRI T2* hati (r = -0,357; p = 0,016). Simpulan: Terdapat korelasi antara muatan besi berlebih (feritin serum dan LIC) dengan nilai elastografi hati. Terdapat korelasi negatif nilai elastografi hati dengan MRI T2* hati pada pasien thalasemia intermedia dewasa.
Kata Kunci: Thalasemia Intermedia, Feritin Serum, MRI T2* Hati, LIC, Elastografi Hati.


Background: Iron overload is a common feature of thalassemia intermedia due to regular blood transfusion and increase gastrointestinal iron absorption. Early detection and adequate iron chelator can significantly decrease related morbidities and mortality due to complication from iron overload. Liver Iron Concentration (LIC) is the best way to measure body iron stores. MRI T2* as a validated test to identify LIC, is expensive and currently not available in all medical services in Indonesia. Objective: To identify liver iron overload and correlation of transferrin saturation, serum ferritin, liver MRI T2*, and LIC with transient liver elastography in adult thalassemia intermedia patient. Methods: We conducted a cross sectional study enrolling 45 patients with thalassemia intermedia with blood transfusion and with and without iron chelator therapy. The study was conducted at Cipto Mangunkusumo Hospital from August 2016 through October 2016. We performed measurements of transferrin saturation, serum ferritin level, transient liver elastography and liver MRI T2*. The Pearson and Spearman correlation test were used to evaluate the correlation transient liver elastography with transferrin saturation, serum ferritin, Liver MRI T2*, and LIC. Results: As much as 64,4% of study subject are β-Hb E Thalasemia Intermedia with median (IQR) age is 33 (22) years old. As much as 84,4% of study subject have regular blood transfusion. On the basis of liver MRI T2*, all studi subject suffered from liver iron overload, with 48,9% had severe liver iron overload. The median value of Liver MRI T2* was 1,6 ms. The mean serum ferritin was 2831 (1828) ng/mL, with median value of transferrin saturation was 66%. The mean of LIC corresponding to Liver MRI T2* and mean liver stiffness measurement was 15,36 ± 7,37 mg Fe/gr dry weight and 7,7 ± 3,8 Kpa respectively. Liver Stiffness correlated with serum ferritin ( r = 0,651; p = 0,000), Liver MRI T2* (r = -0,357; p = 0,016), and LIC ( r = 0,433; p = 0,003). No correlation was found between liver elastography and transferrin saturation ( r = 0,204; p 0,178). Conclusions: Serum ferritin, Liver MRI T2*, and LIC correlated with liver elastography. No correlation was found between transferin saturation and liver elastography.
Key Words: Thalasemia Intermedia, Serum ferritin, Liver MRI T2*, LIC, Transient Liver Elastography.