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Hikari Ambara Sjakti
"Pasien leukemia mieloblastik akut (LMA) yang mencapai remisi komplet pascaterapi induksi di Indonesia hanya 49%, dan event-free survival (EFS) hanya 10%. Angka kekambuhan dan kematian terkait kemoterapi menjadi penyebab rendahnya luaran tersebut. Untuk meningkatkan luaran dan mengurangi efek samping pengobatan perlu dilakukan stratifikasi risiko menggunakan profil sitogenetik maupun imunofenotipe. Tujuan penelitian ini adalah mendapatkan profil imunofenotipe, kariotipe, mutasi FLT3-ITD dan NPM1 (variabel prognosis), serta hubungannya dengan respons kemoterapi induksi.
Penelitian dilakukan dengan desain kohort analitik pada LMA usia 1–18 tahun di RSCM, RSABHK, RSKAD, RSPAD pada bulan November 2018 hingga Maret 2020. Pemeriksaan yang dilakukan meliputi ekspresi CD7, CD19, kariotipe t(8,21), inv(16), mutasi NPM1 dan FLT3-ITD, kemudian dinilai hubungannya dengan kejadian remisi setelah mendapat terapi induksi dengan protokol LMA Nasional.
Dari 42 subjek diperoleh median usia 8 tahun 11 bulan (3–213 bulan). Tipe LMA terbanyak adalah M1, diikuti M2. Gejala klinis tersering pucat (33/42) dan demam (25/42). Tanda klinis terbanyak hepatomegali (17/42) dan splenomegali (18/42). Subjek dengan CD7+ 21,4%, CD19+ 11,9%. Translokasi t(8;21) terdeteksi pada 1dari 18 (5,6%) subjek, inv(16) pada 4 dari 18 ((22%) subjek, 7 dari 18 subjek termasuk kelompok kariotipe favorable. Sebanyak 2 dari 28 (7%) subjek memiliki mutasi FLT3-ITD. Mutasi NPM1 tidak ditemukan. Ekspresi CD7 lebih dominan berperan dibandingkan usia dan jumlah leukosit saat diagnosis sebagai faktor prognosis baik. Analisis multivariat menunjukkan hubungan bermakna antara variabel prognosis dengan respons terapi induksi. Aberans CD7, inv(16) dan mutasi FLT3-ITD memiliki risiko relatif lebih tinggi untuk remisi (masing-masing incidence rate ratio/IRR 3,39 (IK 95% 1,43–8,04); IRR 2,36 (IK 95% 1,08–5,17); dan IRR 4,08 (IK 95% 1,78–9,34)). Nilai IRR aberans CD19 dan t(8;21) IRR < 1.
Penelitian ini menunjukkan aberans CD7, inv(16) dan mutasi FLT3-ITD dapat dijadikan faktor prognosis baik sedangkan aberans CD19, kariotipe t(8;21) dapat dijadikan faktor prognosis buruk pada LMA anak di Indonesia.

Currently, pediatric acute myeloid leukemia (AML) patients who achieved complete remission after induction therapy has reach only 49% with 10% event-free survival (EFS) rate. The relapse rate and mortality related to chemotherapy are the causes of this low outcome. To improve outcomes and reduce side effects of treatment, it is necessary to carry out risk stratification using cytogenetic profiles and immunophenotypes. The purpose of this study was to obtain profiles of immunophenotype, karyotype, FLT3-ITD and NPM1 mutations (prognostic variables), and their relationship to the response to induction chemotherapy.
The study was conducted with an analytical cohort design on AML patients aged 1–18 years at RSCM, RSABHK, RSKAD, RSPAD from November 2018 to March 2020. The examinations included expression of CD7, CD19, karyotype t(8.21), inv(16), NPM1 and FLT3-ITD mutations, then assessed the relationship with the incidence of remission after receiving induction therapy with the National AML protocol.
Of the 42 subjects, the median age was 8 years 11 months (3–213 months). The most common type of AML was M1, followed by M2. The most common clinical symptoms were pallor (33/42) and fever (25/42). The most common clinical signs were hepatomegaly (17/42) and splenomegaly (18/42). Subjects with CD7+ 21.4%, CD19+ 11.9%. Translocation t(8;21) was detected in 1 of 18 (5.6%) subjects, inv(16) in 4 of 18 ((22%) subjects, 7 of 18 subjects included in the favorable karyotype group. A total of 2 of 28 (7%) subjects had FLT3-ITD mutations. NPM1 mutation was not found. Role of CD7 expression as prognostic factor was more dominant than age and leukocyte count at diagnosis. Multivariate analysis using generalized linear model showed a significant relationship between prognostic variables and response to induction therapy. CD7 aberrant, inv(16) and FLT3-ITD mutations had a higher relative risk for remission (respectively incidence rate ratio /IRR 3.39 (95% CI 1.43–8.04); IRR 2.36 (95% CI 1.08–5.17); and IRR 4.08 (95% CI 1.78–9.34)). Incidence rate ratio value of CD19 aberrant and t(8;21) IRR < 1.
This study showed that CD7 aberrant, inv(16) and FLT3-ITD mutations can be used as good prognostic factors, while CD19 aberrant, t(8;21) karyotype can be used as poor prognostic factors for pediatric AML in Indonesia.
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Jakarta: Fakultas Kedokteran Universitas Indonesia, 2022
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UI - Disertasi Membership  Universitas Indonesia Library
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Nora Sovira
"Akumulasi rantai globin-α berlebihan pada membran SDM thalassemia-β mayor menyebabkan hemolisis, eritropoiesis tidak efektif dan anemia kronik sehingga memerlukan transfusi sel darah merah (SDM) terus menerus. Transfusi rutin dan hemolisis mengakibatkan besi bebas sebagai radikal bebas dan membentuk radikal peroksil lipid di membran SDM sehingga memperberat hemolisis. Antioksidan α-tokoferol menghambat pembentukan radikal peroksil lipid tersebut.
Penelitian ini bertujuan untuk menilai peran α-tokoferol terhadap hemolisis dan stres oksidatif. Penelitian ini merupakan uji klinis acak tersamar ganda pada thalassemia-β mayor usia 5–18 tahun yang mendapat transfusi dan kelasi besi rutin di Pusat Thalassemia RSUP dr.Ciptomangunkusumo Kiara. Intervensi plasebo dan α-tokoferol diberikan selama empat minggu. Suplementasi α-tokoferol berdasarkan rekomendasi Institute of Medicine (IOM), 4–8 tahun 200 mg/hari; 9–13 tahun 400 mg/hari; 14–18 tahun 600 mg/hari. Penilaian penanda hemolisis menggunakan haptogobin (Hp), hemopeksin (Hx) dan fragilitas osmotik SDM. Penanda stres oksidatif yaitu MDA, GSH, GSSG, rasio GSH/GSSG dan α-tokoferol. Pemeriksaan laboratorium dilakukan sebelum dan setelah diberikan plasebo/α-tokoferol, sesaat sebelum transfusi SDM. Analisis uji t-tidak berpasangan untuk melihat perbedaan antara kelompok studi dan uji korelasi untuk melihat hubungan antara variabel.
Pada bulan Desember 2016 hingga Juli 2017, 40 subjek mampu menyelesaikan penelitian, 20 subjek kelompok plasebo dan 20 subjek kelompok α-tokoferol. Nilai rerata Hp lebih besar pada kelompok α-tokoferol (3,01 mg/dL) dibandingkan kelompok plasebo (1,08 mg/dL), secara statistik berbeda bermakna (p = 0,021). Nilai rerata kadar Hx dan persentase hemolisis SDM tidak berbeda bermakna pada kedua kelompok studi (p > 0,05). Tidak ada perbedaan bermakna pada kelompok α-tokoferol dan plasebo untuk kadar MDA (1,003 nmol/L dan 1,07 nmol/L), GSH (5,81 µM dan 6,15 µM), GSSG (1,77 µM dan 1,86 µM) dan rasio GSH/GSSG (1,29 dan 1,31), (P > 0,05).
Antioksidan α-tokoferol dapat mengurangi hemolisis dan secara tidak langsung memperbaiki kadar Hp pada thalassemia-β mayor, akan tetapi tidak mampu memengaruhi stres oksidatif.

The accumulation of excess unmatchedα-globin chains in the red blood cell membrane of β-thalassemia major leads to hemolysis, ineffective erythropoiesis and chronic anemia which needs multiple red blood cell transfusion. Routine transfusions may lead to iron overload as free radical in the red blood cell membrane, resulting clinically as severe hemolysis. Alpha-tocopherol as an antioxidant has been known as a potent scavenger of hydroxyl lipid radical.
The aim of this study was to evaluate the effects of α-tocopherol in hemolysis and oxidative stress on the red cell membrane in β-thalassemia major. This randomized double-blind, placebo-controlled study was done in β-thalassemia major patients range aged 5–18 years old who regularly had transfusion and receiving iron chelating agents at Thalassemia centre, Kiara Ciptomangunkusumo Hospital. All subjects were randomized to receive either α-tocopherol or placebo orally for 4 weeks. Subjects in the experimental group received α-tocopherol, the doses based on the recommendation from Institute of Medicine (IOM) as follows: 200 mg/day for 4–8 years old; 400 mg/day for 9–13 years old; 600 mg/day for 14–18 years old. Laboratory analysis for hemolysis variables were haptoglobin, hemopexin, osmotic fragility test. Oxidative stress and antioxidant variables were MDA, GSH, GSSG, GSH/GSSG ratio, and α-tocopherol. All variable were evaluated before 4 weeks and after consuming α-tocopherol or placebo, just before they received a blood transfusion. The statistical analysis results using independent t-test and correlation test.
During December 2016–July 2017, 40 subjects completed the study, they were 20 subjects in the placebo group and 20 subjects in the α-tocopherol group. There was significant enhancement of haptoglobin mean level in the α-tocopherol group (3.01 mg/dL) compared to placebo (1.08 mg/dL), (p = 0.021). The mean level of hemopexin and the percentage of RBC hemolysis did not significantly different in both groups, (p > 0.05). We also did not find any significantly different in mean level of MDA (1.003 nmol/L and 1.07 nmol/L), GSH (5.81 µM and 6.15 µM), GSSG (1.77 µM and 1.86 µM) and GSH/GSSG ratio (1.29 and 1.31), (p > 0.05) for the α-tocopherol and placebo groups.
The effects of α-tocopherol may improve hemolysis and haptoglobin level indirectly in β-thalassemia major, but there was no significant role in oxidative stress."
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Depok: Fakultas Kedokteran Universitas Indonesia, 2019
D-Pdf
UI - Disertasi Membership  Universitas Indonesia Library