Tesis

Faktor Hemolisis Dan Akumulasi Zat Besi Yang Berhubungan Dengan Probabilitas Hipertensi Pulmonal Pada Pasien Talasemia = Hemolysis and Iron Accumulation Factors Associated with Probability of Pulmonary Hypertension in Thalassemia Patient.

Latar Belakang: Jumlah penderita talasemia dari tahun ke tahun selalu meningkat. Salah satu komplikasi penting talasemia adalah gangguan kardiovaskuler, di antaranya adalah hipertensi pulmonal Pemeriksaan kateter jantung kanan dan ekokardiaografi tidak dapat dilakukan pada tahap awal, sehingga diagnosis hipertensi pulmonal sering terlewat. Mengingat hipertensi pulmonal merupakan penyebab kematian pada pasien talasemia dewasa maka diperlukan pemeriksaan yang bisa mengetahui lebih awal kejadian hipertensi pulmonal. Beberapa faktor, yaitu bilirubin, retikulosit, LDH, dan ferritin diteliti hubungannya dengan kejadian hipertensi pulmonal. Tujuan: Mengetahui proporsi hipertensi pulmonal dan faktor-faktor yang berhubungan dengan probabilitas kejadian hipertensi pulmonal pada pasien talasemia di RSCM. Metode: Penelitian ini dilakukan dengan desain kohort restrospektif dan konsekutif sampling. Penelitian mengambil semua pasien talasemia dewasa (di atas 18 tahun) yang mendapatkan transfusi rutin dari bulan Juli 2023 – September 2024 di poliklinik talasemia dewasa RSCM Jakarta. Kemudian dilakukan pengumpulan data melalui rekam medis atau pemeriksaan langsung. Data yang diperoleh diolah menggunakan SPSS. Hasil: Sebagian besar subjek penelitian adalah perempuan sebanyak 42 orang (68,9) dan diagnosis terbanyak adalah talasemia beta/HbE sebanyak 38 orang (62,3). Penelitian ini juga menunjukkan proporsi hipertensi pulmonal pada subjek terdapat 8 orang (13,1%) yang termasuk kategori high risk dan 6 orang (75%) di antaranya termasuk talasemia beta/HbE. Penelitianini juga menunjukkan tidak terdapat perbedaan rerata antara kelompok high risk dan low risk pada feritin (7.761:7.268), bilirubin (2,26:1,78), retikulosit(1,59:1,46), dan LDH (185:171). Hasil uji korelasi kadar serum feritin dengan TRV bermakna dengan nilai r 0,254 dan p 0,048. Simpulan: Proporsi kejadian hipertensi pulmonal pada talasemia dewasa di RSUPN Cipto Mangunkusumo pada penelitian ini sebesar 13,1%. Terdapat hubungan antara serum feritin dengan probabilitas hipertensi pulmonal, namun tidak terdapat hubungan antara bilirubin, retikulosit dan LDH terhadap hipertensi pulmonal pada penderita talasemia.
Kata kunci: Talasemia, Hipertensi Pulmonal, Feritin, Bilirubin, Retikulosit, LDH


Background: The number of thalassemia sufferers from year to year is always increasing. One of the important complications of thalassemia is cardiovascular disorders, including pulmonary hypertension. Right heart catheter examination and echocardiography cannot be performed at an early stage, so the diagnosis of pulmonary hypertension is often missed. Given that pulmonary hypertension is the cause of death in adult thalassemia patients, an examination is needed that can detect the occurrence ofpulmonary hypertension early. Several factors, including bilirubin, reticulocytes, LDH, and ferritin have been studied for their relationship on theoccurrence of pulmonary hypertension. Objective: To determine the proportion of pulmonary hypertension and factors that associated with the probability of pulmonary hypertension in thalassemia patients atRSCM. Methods: This study was conducted using a retrospective cohort design with consecutive sampling. All adult thalassemia patients (aged 18 years and above) who received routine transfusions from July 2023 to September 2024 at the adult thalassemia clinic ofRSCM, Jakarta, were included. Data were collected through medical records or direct examination. The collected data were analyzed using SPSS. Results: The majority of the subjects were female (42 people, 68.9%) and the most common diagnosis was beta-thalassemia/HbE (38 people, 62.3%). This study also revealed that 8 subjects (13.1%) had pulmonary hypertension, categorized as high risk, and 6 of them (75%) were diagnosed with beta-thalassemia/HbE. Furthermore, this study also showed that there was no difference in the mean between the high-risk and low-risk groups in ferritin (7.761:7.268), bilirubin (2,26:1,78), reticulocytes (1,59:1,46), and LDH (185:171). The result of correlation test between ferritin serum level and TRV were significant with a r value of 0,254 and p 0,048. Conclusions: The prevalence of pulmonary hypertension in adult thalassemia patients at RSUPN Cipto Mangunkusumo in this study was 13.1%. There is a relationship between serum ferritin and the probability of pulmonary hypertension, but there is no relationship between bilirubin, reticulocytes and LDH on pulmonary hypertension in thalassemia sufferers.
Key word: Thalassemia, Pulmonary Hypertension, ferritin, bilirubin, reticulocytes, LDH