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"Seratus lima belas penderita thalassemia beta mayor yang mendapatkan tranfusi rutin di Pusat Thalassemia Bagian Ilmu Kesehatan Anak, Fakultas Kedokteran, Universitas Indonesia ? Rumah Sakit Umum Pusat Dr. Cipto Mangunkusumo dilakukan pemeriksaan hematologi. Didapatkan korelasi yang negatif antara besarnya limpa dengan parameter hematologi darah tepi. Hasil pemeriksaan parameter darah tepi cenderung menurun dengan membesarnya limpa dan kondisi tersebut akan membaik setelah splenektomi. Pada penelitian kami hipersplenisme dimulai pada limpa S (V ? VI). Pemeriksaan elektroforesis hemoglobin didapatkan penebalan fraksi HbF dan 90 penderita tersebut menunjukkan pola yg normal. Oleh karena itu, untuk melakukan konfirmasi elektroforesis hemoglobin perlu dilakukan dengan pemeriksaan elektroforesis hemoglobin pada kedua orang tua atau pemeriksaan analisis genetik dengan teknik biomolekuler. (Med J Indones 2004; 13: 8-16)

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One hundred and fifteen beta thalassemia major outpatients attending the Thalassemia Center Department of Child Health, Medical School University of Indonesia Dr. Cipto Mangunkusumo General Hospital for routine blood transfusion and hematology examination, participated in this study. There was a negative correlation between the size of the spleen and the peripheral blood parameters. All peripheral blood parameters tend to decrease with the enlargement of the spleen, and the condition is reversed after splenectomy. We observed that hypersplenism starts when the spleen is as big as S (V ? VI). The hemoglobin electrophoresis pattern from beta thalassemia major patients receiving repeated blood transfusion did not show a dense HbF fraction, 90 patients showed a normal hemoglobin electrophoresis pattern. A hemoglobin analysis of both parents could be useful to confirm the diagnosis of beta thalassemia major for patients receiving repeated blood transfusion. In order to get a definite diagnosis, a genetic analysis by bio molecular technique is needed. (Med J Indones 2004; 13: 8-16)"

"Thalassemia b mayor adalah penyakit yang disebabkan oleh kelainan sintesis rantai polipeptida b yang diturunkan secara otosom resesif. Pengobatan thalassemia b mayor pada umumnya berupa pemberian transfusi berulang, yang mengakibatkan penumpukan besi dan berakhir dengan hemokromatosis. Penumpukan besi dapat terjadi pada organ tubuh antara lain ginjal. Tujuan penelitian ini untuk mengetahui adanya gangguan fungsi ginjal pada penderita thalassemia b mayor berumur 15-28 tahun yang telah mendapatkan 6 unit packed red cells. Pada penelitian ini telah diperiksa kadar besi serum (SI) dan daya ikat besi total (TIBC) serta kadar mikroalbumin dan b2-mikroglobulin (b2-m) dalam urin. Hasil yang didapat 94,7% penderita menunjukkan peningkatan saturasi transferin dan 40% diantaranya disertai hemokromatosis; 73,4% disertai mikroalbuminuria, 1,3% dengan albuminuria dan 21,3% dengan peningkatan b2-m urin. Jumlah kasus dengan kelainan ginjal dijumpai pada 78,6%. (Med J Indones 2003; 12: 215-223)

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b-thalassemia major is a disease caused by b polypeptide chain synthesis disorder which is inherited in an autosomal recessive manner from both parents and which is marked by little or no b-globin chain synthesis. Treatment for b-thalassemia major patients is by giving repeated blood transfusions, which causes iron accumulation, leading to hemochromatosis. Iron accumulation can occur in various body organ, including the kidneys. The aim of this study was to investigate the existence of renal impairment in b-thalassemia major patients. The subjects of this study were b-thalassemia major patients aged 15 - 28 years old who had received 6 units of packed red cells or more within 6 months. In this study, urine and serum samples of the subjects were taken and examined. Assay of serum iron was performed with Hitachi 737. Results were that 94.7% patients showed an increase in transferrin saturation and 40% of them had hemochromatosis; 73.4% had microalbuminuria; 1.3% had albuminuria and 21.3% had increased urinary b2-microglobulin (b2-m). A total of 78.6% of patients showed renal impairment. Conclusion of this study suggested that glomerular dysfunction happens in an earlier stage of the disease process. The high incidence of microalbuminuria is also attributed to defective ability of the proximal tubular cells to reabsorb protein besides dysfunction of the glomeruli. (Med J Indones 2003; 12: 215-223)"

"Latar Belakang: Pencapaian target transfusi darah pada pasien thalassemia beta bergantung transfusi dipengaruhi oleh berbagai faktor diantaranya adalah genotip, hipersplenisme, kompatibilitas darah, kecukupan darah donor dan interval transfusi. Ukuran limpa dapat dijadikan salah satu indikator keberhasilan pencapaian target transfusi darah selain kadar hemoglobin.Tujuan: Mengetahui proporsi pasien yang mencapai target optimal kadar hemoglobin pra dan pascatransfusi, menentukan faktor-faktor yang terkait dengan pencapaian target kadar hemoglobin pra dan pascatransfusi dan menilai hubungan antara pencapaian target kadar hemoglobin pra dan pascatransfusi dengan ukuran limpa pada pasien dewasa thalassemia beta bergantung transfusi.Metode: Penelitian cohort retrospective dengan pengambilan 200 subjek secara total sampling pada pasien dewasa rawat jalan Poliklinik thalassemia Rumah Sakit Cipto Mangunkusumo. Data dianalisis dari 110 subjek berupa anamnesis, pemeriksaan fisis dan laboratorium.Hasil: Sebanyak 200 pasien thalassemia beta bergantung transfusi yang rutin kontrol ke poliklinik thalassemia Kiara RSCM, diikuti secara kohort sejak bulan Juni 2017 sampai Juni 2018. 110 subjek penelitian memenuhi kriteria inklusi diantaranya subjek thalassemia beta mayor 53 (48,2%) dan beta HbE bergantung transfusi 57 (51,8%). Proporsi subjek yang mencapai target kadar Hb pratransfusi yaitu 18 (16,4%) dan 22 (20,0%) subjek yang mencapai target Hb pasca. Sebanyak 8 (7,3%) subjek mencapai target kadar Hb pra dan pascatransfusi darah. Faktor kecukupan darah donor berhubungan dengan pencapaian target kadar Hb pra dan pascatransfusi (p=0,008) yaitu subjek yang hanya memiliki selisih permintaan darah < 30ml/KgBB/tahun. Pada 93 subjek penelitian tahap 2, didapatkan perbedaan bermakna antara kelompok yang tercapai kadar Hb pra dan pascatransfusi darah dengan yang tidak tercapai terhadap delta ukuran limpa (p <0,001).Simpulan: Faktor kecukupan darah donor berhubungan dengan pencapaian target kadar hemoglobin pra dan pascatransfusi. Pencapaian target kadar hemoglobin pra dan pascatransfusi berhubungan dengan ukuran limpa.

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Background: Achieving the target of blood transfusion in transfusion-dependent beta thalassemia patients is influenced by various factors including genotype, hypersplenism, blood compatibility, donor blood adequacy and transfusion interval. The size of the spleen can be one indicator of the success of achieving blood transfusion targets in addition to hemoglobin levels.

Objective: Determine the proportion of patients who achieve the optimal target hemoglobin level pre and post transfusion, determine the factors that are related to achieving pre and post transfusion hemoglobin levels and assess the relationship between achieving pre and post transfusion hemoglobin levels with spleen size in adult beta thalassemia transfusion dependent patients.

Methods: A cohort retrospective study, with total sampling of 200 adult thalassemia transfusion dependent patient at Cipto Mangunkusumo Hospital. Data taken from 110 eligible subject in the form of medical history, physical examination and laboratory.

Result: 200 transfusion-dependent beta thalassemia patients who routinely visit the RSCM thalassemia Kiara polyclinic, followed in cohort from June 2017 to June 2018. 110 study subjects fulfilled the inclusion criteria including 53 (48.2%) major beta thalassemia subjects and transfusion-dependent HbE beta 57 (51.8%). The proportion of subjects who achieved pre-transfusion Hb target levels was 18 (16.4%) and 22 (20.0%) subjects who achieved the post Hb target. A total of 8 (7.3%) subjects achieved pre and post transfusion Hb levels. The donor blood adequacy factor is related to the achievement of pre and post transfusion Hb target levels (p = 0.008), namely subjects who only have a blood demand difference of <30ml/KgBB/year. In 93 research subjects, there was a significant difference between groups who achieved pre and post-transfusion Hb levels with those that were not reached against the delta of spleen size (p <0.001).

Conclusion: Adequacy factor of donors blood is related to achieving target pre and post transfusion hemoglobin levels. The achievement of the target pre and post transfusion hemoglobin levels is related to the size of the spleen."

"ABSTRAKLatar Belakang: Muatan besi berlebih yang diduga dapat menyebabkan peningkatan stres oksidatif malondialdehyde merupakan masalah utama pada pasien thalasemia beta mayor dan intermedia, baik TDT maupun NTDT. Transfusi darah dan kelasi besi merupakan terapi utama thalasemia. Kadar malondialdehyde MDA plasma belum diteliti mendalam di Indonesia, terutamapada pemberian transfusi serta korelasinya dengan muatan besi berlebih.Tujuan: mendapatkan profil kadar MDA pada pasien thalasemia dewasa; membandingkan kadarnya antara sebelum dan setelah transfusi antara pasien TDT dan NTDT serta mendapatkan korelasinya dengan feritin serum FS dan saturasi transferin ST .Metode: Penelitian potong lintang serta pre dan post study pada penderita dewasa thalasemia beta yang mendapatkan transfusi darah serta dengan/atau tanpa kelasi besi. Sampel darah diambil sesaat sebelum transfusi dan satu hari setelah transfusi. Kadar MDA plasma diperiksa berdasarkan metode Wills.Hasil: Sebanyak 63 orang pasien dilibatkan dalam studi, terdiri dari 51 TDT dan 12 NTDT. Median kadar MDA adalah 0,49 0,21-1,33 ?mol/L. Kadar tersebut tidak berbeda bermakna antara sebelum dan setelah transfusi, antara pasien TDT dan NTDT. Didapatkan korelasi lemah antara FS dengankadar MDA sebelum transfusi sedangkan tidak ada korelasi antara FS dengan kadar MDA setelah transfusi dan antara ST dengan kadar MDA.Simpulan: Median kadar MDA plasma pada pasien dewasa dengan thalasemia beta mayor dan intermedia sebelum transfusi 0,49?mol/L.Tidak ada perbedaan bermakna antara kadar MDA sebelum dan setelah transfusi dan antara pasien TDT dan NTDT. Terdapat korelasi lemah antara FS dengan MDA sebelum transfusi dan tidak terdapat korelasi FS dengan MDA setelah transfusidan ST dengan kadar MDA.

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ABSTRACTAbstract Background Iron overload is a major problem in thalassemic patients, either in TDT or NTDT. Iron overload may increase oxidative stress malondialdehyde MDA . Blood transfusion and chelating iron are the main therapy for beta thalassemia major TDT. However, plasma MDA levels have not been well studied in Indonesia, especially its correlation with iron overload.Objective This study aimed to profile MDA levels in adult thalassemic patients to compare its level between before and after transfusion and between TDT and NTDT patients and to obtains its correlation with serum ferritin SF and transferrin saturation TS .Method A cross sectional as well as pre and post study in adult patients with thalassemia major and intermedia who received blood transfusion with or without chelating iron.Blood samples were withdrawn immediately before transfusion and one day after transfusion. Plasma MDA levels were assayed according to Wills method. Results Total of 63 patients were enrolled, consisting 51 TDT and 12 NTDT patients. Median MDA level was 0.49 0,21 1,33 mol L. The level was not significantly different between before and after transfusion, between TDT and NTDT patients. Weak correlation was observed between SF and MDA levels before transfusion and there is no correlation was observed between SF and MDA levels before transfusion and also between TS and MDA levels.Conclusion Median plasma MDA levels in adult patients with beta thalassemia major and intermedia before transfusion 0,49 mol L. No significant different is found between MDA before and after transfusion and between TDT and NTDT patients as well. Plasma MDA levels have weak correlation with serum feritin levels before blood transfusion."

"Latar belakang: Thalassemia merupakan kelainan hemoglobinopati yang cukup banyak di Indonesia. Terapi utama thalassemia mayor adalah transfusi seumur hidup. Transfusi berulang memiliki efek samping. Salah satunya adalah terbentuknya aloantibodi sel darah merah. Prevalens dan faktor-faktor yang memengaruhi aloantibodi pada pasien thalassemia masih belum ada di Indonesia. Uji Coombs sebagai standar diagnosis merupakan pemeriksaan yang mahal dan hanya tersedia di pusat tertentu. Metode lain yang lebih mudah diperlukan untuk memprediksi terbentuknya aloantibodi tersebut. Tujuan: Untuk mengetahui prevalens aloantibodi sel darah merah di populasi Indonesia dan mendapatkan faktor-faktor yang memengaruhinya. Membuat sistem skoring untuk memprediksikan probabilitas terbentuknya aloantibodi sel darah merah berdasarkan faktor-faktor tersebut. Metode: Analisis terhadap 162 rekam medis subjek yang telah dilakukan uji Coombs di Pusat Thalassemia Jakarta pada tahan 2005-2013. Hasil: Dari 162 subjek didapatkan 31 (19%) subjek memiliki aloantibodi dan 4 (2,4%) subjek menderita AIHA. Jenis aloantibodi terbanyak yang terdeteksi adalah anti-M (29%). Faktor-faktor yang memengaruhi terbentuknya aloantibodi adalah tingginya volume transfusi, jarak antar transfusi, lama transfusi, kadar leukosit dan pajanan PRC biasa. Berdasarkan faktor-faktor risiko tersebut, sistem skoring didisain untuk memprediksi kemungkinan terbentuknya aloantibodi. Kesimpulan: Prevalens aloantibodi pada pasien thalassemia di Indonesia cukup tinggi. Pemberian PRC leukodeplesi pelu direkomendasikan pada pasien dengan transfusi berulang. Prediksi terbentuknya aloantibodi dapat dilakukan melalui sistem skoring terutama di tempat yang tidak tersedia uji Coombs.

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Background: Thalassemia major is a common genetic disease in Indonesia. The principal treatment of thalassemia major is lifelong blood transfusion, which is frequently complicated by alloantibody. Limited data are available on the frequency of RBC alloantibody and factors influencing in major β-thalassemia patients. Coombs test, as a standard tool to diagnose alloantibody, is only available on particular Red Cross Centre. Therefore, it is necessary to find another tool to predict the probability of alloantibody formation.

Aim: To investigate the prevalence of RBC alloantibody among thalassemia major patients in Thalassemia Centre Jakarta. To describe factors influencing RBC alloantibody production and develop scoring system to predict its probability.

Methods: We analyzed the clinical and transfusion records of 162 thalassemia major patients who have been examined for Coombs test. All of the patients were registered in Thalassemia Center, Cipto Mangunkusumo hospital from 2005 until 2013.

Results: Of the 162 subjects, 31 (19%) developed RBC alloantibody and four patients (2,4%) developed autoimmune hemolytic anemia. The most common alloantibody was anti-M (29%).Several factors were found to contribute to high alloantibody rate in this study, including high volume of transfusion, duration of transfusion, white blood count level, transfusion interval, and PRC exposure. From those factors, scoring system has been developed to predict alloantibody formation in thalassemia patients.

Conclusion: We concluded that there is a high rate of RBC alloantibody in major thalassemia patients in our center. We also suggest that leukocyte-poor PRC should be given to all patients with multiple transfusions. In remote area where Coombs test is not available, scoring system can be used to predict the probability of alloantibody formation."

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Latar Belakang: Setiap individu memiliki antigen sel darah merah (SDM) yang unik pada membrannya. Terdapat lebih dari 300 antigen SDM yang dapat membagi darah ke dalam 36 sistem golongan. Adanya variasi pada antigen sel darah merah menyebabkan uji kecocokan darah antara pasien dengan donor wajib dilakukan guna mencegah terjadinya reaksi antara antigen donor dengan antibodi pasien. Pasien thalassemia memerlukan transfusi darah rutin yang dapat meningkatkan risiko terbentuknya aloantibodi, sehingga seringkali sulit untuk menemukan darah donor yang kompatibel. Unit Transfusi Darah (UTD), dalam rangka menjamin keselamatan pasien, harus mampu menyediakan darah donor tanpa antigen yang dapat menyebabkan reaksi transfusi. Pemeriksaan genotipe akan memberikan gambaran variasi antigen SDM donor, sehingga memudahkan pencarian donor yang sesuai untuk resipien.

Tujuan: Mengetahui adanya variasi genotipe antigen SDM pada donor sehingga dapat diupayakan penyerasian antigen darah donor untuk pasien transfusi berulang.

Metoda: Penelitian ini merupakan penelitian deskriptif observasional dengan desain potong lintang. Subyek pada penelitian ini adalah donor untuk pasien thalassemia. Sampel darah donor dikumpulkan untuk dilakukan pemeriksaan golongan darah ABO, Rhesus, ekstraksi DNA, dan genotipe antigen SDM.

Hasil dan Diskusi: Dari total 161 subyek penelitian, distribusi ABO/Rhesus donor adalah 68 subyek O+(42,24%), 43 subyek A+, 41 subyek B+, dan 9 subyek AB+. Setelah dilakukan pemeriksaan genotipe antigen SDM, didapatkan golongan darah dengan genotipe tersering untuk masing-masing golongan darah sebagai berikut Ce (98%) pada Rhesus, k/k (100%) pada Kell, Jk^a/Jk^b (40,76%) pada Kidd, Fy^a/Fy^a (74,84%) pada Duffy, Di^b/Di^b (99,36%) pada Diego, Do^b/Do^b (80,89%) pada Dombrock, Co^a/Co^a (100%) pada Colton, Yt^a/Yt^a (98.09%) pada Cartwright, MN (47,37%), s (86,54%) pada MNS dan Lu^b/Lu^b (100%) pada Lutheran. Pada studi ini juga ditemukan beberapa antigen darah langka seperti cE (1,33%), cEe (2%), CEe (1,33%), Fy^b (1,29%), Di^aDi^b (0,64%), Yt^aYt^b (1,91%), dan S (1,94%). Perlu diperhatikan antigen darah langka yang ditemukan pada donor/ populasi umum, bila ditransfusikan kepada pasien dengan antigen umum, dapat memicu timbulnya antibodi.

Kesimpulan: Pada penelitian ini ditemukan beberapa variasi genotipe antigen golongan darah pada donor, termasuk antigen langka. Perbedaan variasi antigen sel darah merah donor dan pasien dapat menyebabkan timbulnya aloantibodi, terutama pada pasien transfusi berulang. Oleh karena itu, pemeriksaan genotipe antigen SDM diharapkan dapat mengurangi reaksi transfusi dan meningkatkan keamanan pasien, terutama pasien yang membutuhkan transfusi berulang.

Kata kunci: antigen sel darah merah, genotipe, donor, aloantibodi

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Background: Every individual has unique antigens on their red blood cells surface. There are more than 300 antigens of red blood cells that differentiate blood into 36 blood group systems. Due to the variation in antigen of red blood cell, it is a must to perform blood group matching between the patients and donors blood to prevent reactions between the donors antigen and patients antibody in the blood. Thalassemia patients require regular transfusions which resulting in the production of alloantibody, hence making it difficult to find compatible blood. Blood Transfusion Units (UTD) is required to provide blood without antigen that can trigger transfusion reaction to ensure patient safety. Red blood cell antigen genotyping from the donor can describe the variation of red blood cell antigen from the donor.

Aims: To identify the genotype variation of the donor red blood cells antigen, hence optimizing the donors antigen matching in patients with regular transfusion.

Methods: This was a descriptive observational study with cross sectional design. Subjects in this study were donor for thalassemia patients. Blood samples from donors underwent several examinations, such as the ABO blood type testing, Rhesus testing, DNA extraction, and red blood cell antigen genotyping.

Results and Discussions: From a total of 161 research subjects, the distribution of ABO/Rhesus blood grouping are 68 of O+(42,24%), 43 of A+, 41 of B+, and 9 of AB+. From the red blood cell antigen genotyping, variations of red cell antigens were found in several blood group systems as follows, Rhesus, Kidd, Kell, Duffy, MNS, Diego, Dombrock, Colton, Cartwright, and Lutheran. Our findings also shown several rare antigens such as cE (1,33%), cEe (2%), CEe(1,33%), Fy^b (1,29%), Di^aDi^b (0,64%), Yt^aYt^b (1,91%), S (1,94%). It is important to note that rare blood antigens were found in donors/ general population, if blood is transfused to patients, it can trigger the alloantibody production.

Conclusion: Our study found there were genotype variations in the blood antigen of donor, some of them were rare types. The difference of red blood cell antigen between donors and patients may lead to the development of alloantibody, especially in patients who need multiple transfusion. Therefore, red blood cell antigen genotyping is expected to decrease the incidence of transfusion reactions and increase patient safety, especially in patients that required multiple transfusions.

Keywords: Red blood cells antigen, genotyping, donor, alloantibody

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"Latar belakang. Kadar hemoglobin pre-transfusi dan feritin serum mempengaruhi pertumbuhan anak dengan thalassemia B-mayor. Penelitian tentang thalassemia sudah dilakukan di Indonesia, namun penelitian tentang hubungan thalassemia dengan pertumbuhan fisik masih terbatas. Tujuan. Mengetahui pengaruh kadar Hb pre-transfusi dan feritin serum berpengaruh terhadap pertumbuhan fisik pasien thalassemia ?-mayor. Metode. Dilakukan bulan Agustus-Oktober 2017 pada pasien anak dengan thalassemia B-mayor yang berobat ke Thalassemia-Centre RSUD Pekanbaru. Penelitian berupa analitik observasional potong lintang, menganalisis pengaruh kadar Hb pre-transfusi dan feritin serum terhadap parameter perawakan pendek dan sangat pendek, gizi kurang dan buruk, usia tulang yang terlambat. Hasil. Subjek 41 orang, rentang usia 18-204 bulan. Jenis kelamin laki-laki lebih banyak daripada perempuan 53,7 vs 46,3. 40 subjek mengalami retardasi pertumbuhan. Terdapat korelasi bermakna antara kadar Hb pre-transfusi dengan Z-score TB/U r=0,507, p=0,001 dan LILA/U r=0,467, p=0,02. Hb pre-transfusi berpengaruh terhadap interpretasi duduk/umur p=0,007, IK95 -1,5 - -0,3, subischial leg length/umur p=0,002, namun tidak pada interpretasi rasio segmen atas/bawah dan usia tulang. Hasil berbeda pada kadar feritin yang tidak memiliki korelasi terhadap semua variabel. Simpulan. Terdapat pengaruh yang bermakna secara statistik antara kadar Hb pre-transfusi dengan parameter penelitian serta tidak terdapat pengaruh yang bermakna secara statistik antara kadar feritin serum dengan parameter tersebut.

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Background. The level of pre transfusion hemoglobin and ferritin serum affect physical growth on patient with thalassemic mayor. Study about thalassemia is mainly reported but its relationship with physical growth is limited.

Objective. The main objective of the present study was to evaluate the relationship of pre transfusion Hb and serum ferritin level in patient with thalassemic mayor.

Material and method. In this analytical cross sectional study, the growth parameters weight, standing height, sitting height, subischial leg length, nutritional status, bone age were measured in 41 patients attending Thalassemia Centre at RSUD in Pekanbaru from August October 2017.

Results. 41 patients with mean age 18 204 months. The results are boys dominated girls in sex criteria 53,7 vs 46,3. As much as 40 subjects have growth retardation. There rsquo s correlation in pre transfusion hemoglobin with Z score height for age r 0,507, p 0,001 and subischial length r 0,467, p 0,02. This study shows relationship in pre transfusion hemoglobin with sitting height p 0,007, IK95 1,5 0,3, subischial leg length p 0,002, but not in segment length and bone age. Serum ferritin level has no correlation to one of those parameters.

Conclusion. There is a significant relationship in physical growth based on parameters mentioned above with pre transfusion Hemoglobin, but not with serum ferritin level. "

"Penelitian ini bertujuan untuk mengetahui keluhan subjektif mata kering dan gangguan komponen air mata (lipid, akuos, mucin) pada pasien thalassemia mayor dengan riwayat transfusi darah jangka panjang, serta menganalisis hubungan antara kadar feritin serum, durasi, dan frekuensi transfusi darah dengan masing-masing parameter penilaian komponen lapisan air mata. Penelitian ini merupakan studi potong lintang (cross sectional) pada pasien thalassemia mayor yang sudah berusia dan mengalami transfusi darah selama minimal 10 tahun. Penilaian mata kering terdiri dari pengisian kuesioner OSDI untuk menilai keluhan subjektif, pemeriksaan biomikroskopi lampu celah dan nilai tear break up time (TBUT) untuk menilai tingkat keparahan mata kering, pemeriksaan Schirmer basal, Ferning, dan sitologi impressi konjungtiva untuk menghitung jumlah sel goblet. Data perhitungan tingkat keparahan mata kering, nilai uji Schirmer basal, TBUT, dan jumlah sel goblet dianalisis dan dicari hubungannya dengan kadar feritin serum, durasi dan frekuensi transfusi. Pada 77 subyek, mata kering terjadi sebanyak 14.3%, penurunan nilai TBUT (39%), nilai Schirmer basal (37.7%), nilai Ferning (24.7%), dan jumlah sel goblet (45.5%). Tidak terdapat perbedaan bermakna antara tingkat keparahan mata kering, nilai TBUT, nilai Schirmer basal, nilai Ferning, dan jumlah sel goblet dengan kadar feritin serum, durasi, dan frekuensi transfusi. Namun, terdapat hubungan yang bermakna antara tingkat keparahan mata kering dan usia (p = 0.014), serta nilai TBUT (p = 0.012) dan Schirmer (p = 0.014) dengan jenis kelamin. Penelitian ini memperlihatkan 14.3% subyek thalassemia mayor mengalami mata kering berdasarkan kriteria DEWS 2007. Kejadian mata kering pada thalassemia mayor tidak dipengaruhi oleh faktor transfusi dan kadar feritin serum, melainkan dipengaruhi oleh usia dan jenis kelamin.

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This study is aimed to understand subjective complaints for dry eyes and disruption of component of tear fluid (lipid, aqueous, mucin) in patients with major thalassemia with a history of long-term blood transfusions and to analyse the correlation between serum ferritin level, duration and frequency of blood transfusion. This study is a cross-sectional study. The subject of this study is patients with major thalassemia age minimum of 10 years old and have had blood transfusion for at least 10 years. OSDI questionnaire, slit-lamp biomicroscopy examination, tear break up time (TBUT), and basal Schirmer test was used to assess dry eyes severity. Ferning and conjunctiva impression cytology examination was used to assess mucin quality and count the amount of goblet cells. The correlation analysis between the result of these assessments and serum ferritin level and duration and frequency of blood transfusion was done. In 77 subjects, the prevalence of dry eyes is 14.3%. There is a decrease in TBUT (39%), basal Schirmer (37.7%), Ferning (24.7%), and goblet cells (45.5%). There is no significant correlation between dry eyes severity and TBUT, basal Schirmer, Ferning, and the amount of goblet cells with serum ferritin level, duration, and frequency of blood transfusion. There is a significant correlation between dry eyes severity and patient s age (p = 0.014); TBUT (p = 0.012), as well as, Schirmer (p = 0.014) with sex. This study showed that 14.3% of patients with major thalassemia suffer from dry eyes with severity level grade 2 according to DEWS 2007. The incidence of dry eyes is not influenced by transfusion and serum ferritin level but is influenced by age and sex."