Pustika Amalia Wahidiyat
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Characteristics of Acute Transfusion Reactions and its related factors in Cipto Mangunkusumo Hospital Jakarta, Indonesia Pustika Amalia Wahidiyat; Elida Marpaung; Stephen Diah Iskandar
Health Science Journal of Indonesia Vol 10 No 1 (2019)
Publisher : Sekretariat Badan Penelitian dan Pengembangan Kesehatan

Show Abstract | Download Original | Original Source | Check in Google Scholar | DOI: 10.22435/hsji.v10i1.1847

Abstract

Latar belakang: Reaksi transfusi akut (RTA) merupakan sekelompok kejadian yang tidak diinginkan akibat pemberian transfusi darah. Manifestasi dari RTA bervariasi dari yang ringan hingga mengancam nyawa. Saat ini, data mengenai reaksi transfusi di Indonesia masih sangat terbatas. Dalam studi ini, kami bertujuan untuk memberikan gambaran mengenai karakteristik RTA dan faktor-faktor yang mempengaruhinya. Metode: Studi ini merupakan studi retrospektif yang melibatkan 288 subyek dengan RTA. Studi dilakukan di Rumah Sakit Dr. Cipto Mangunkusumo, dimulai sejak Januari hingga Desember 2017. RTA dikelompokkan berdasarkan sistem tubuh yang mengalami manifestasi, serta derajat manifestasinya. Hasil: Sel darah merah merupakan produk darah utama yang ditransfusikan ke subyek, diikuti dengan konsentrat trombosit, plasma segar beku, dan kriopresipitat. Lima gejala utama dari RTA adalah gatal, demam/kenaikan suhu tubuh, menggigil, urtikaria, dan angioedema. Berdasarkan sistem tubuh yang terkena, umumnya RTA bermanifestasi sebagai gejala pada kulit (56.6%). Berdasarkan derajat manifestasinya, RTA umumnya dikategorikan dalam derajat ringan (55.9%). Anak-anak cenderung mengalami manifestasi yang ringan (64.8%) dan utamanya bermanifestasi pada kulit (65.4%). Riwayat transfusi mempengaruhi derajat RTA secara signifikan. RTA derajat sedang dan gejala konstitusional lebih banyak ditemukan pada subyek yang mendapat PRC dibanding produk darah lainnya. Kesimpulan: Umumnya RTA bermanifestasi sebagai gejala dermatologi. Hanya sedikit kasus RTA yang disebabkan oleh reaksi inkompatibilitas. Manifestasi dan derajat RTA juga dipengaruhi oleh umur, riwayat transfusi, dan jenis komponen darah. Kata kunci: Transfusi darah, reaksi transfusi akut, riwayat transfuse, usia Abstract Background: Acute transfusion reactions (ATRs) are a group of adverse events caused by blood transfusions. Manifestations of ATRs vary from mild to life threatening. At present, data about transfusion reactions in Indonesia are still limited. In this study, we aim to determine the characteristics of ATRs and its related factors. Methods: This was a retrospective study of 288 subjects with ATRs. The study was conducted in Cipto Mangunkusumo Hospital, started from January to December 2017. ATRs were categorized based on the body systems affected and degree of manifestations. Results: Packed red cells (PRC) was the predominant blood product (51.4%) which was transfused to subjects, followed by thrombocyte concentrate (TC), fresh frozen plasma (FFP), and cryoprecipitate. Five most common predominant symptoms of ATRs were pruritus/itch, febrile/increased temperature, chills, transient urticaria, and angioedema. Based on the affected body systems, the majority of ATRs manifested as dermatologic symptoms (56.6%). Based on the degree of manifestations, the majority of ATRs were categorized as mild degree (55.9%). Children tended to have milder symptoms (64.8%), which mostly manifested as dermatologic symptoms (65.4%). History of transfusion affected the degree of ATR significantly. Moderate degree of ATRs and constitutional symptoms were found more common in subjects who received PRC than other blood products. Conclusion: Most of ATRs manifest as dermatologic symptoms, which represent allergic reactions. Only a small portion of ATRs are caused by incompatibility reactions. The manifestation and degree of ATRs are also affected by age, history of transfusion, and type of blood components. Keywords: Blood transfusion, acute transfusion reaction, transfusion history, age
Phenotypic diversity in beta-HbE thalassemia patients Pustika Amalia Wahidiyat; Djajadiman Gatot; Tenny Tjitrasari; Harapan Parlindungan Ringoringo; N S Marzuki; R A Taufani; I Setianingsih; A Harahap
Paediatrica Indonesiana Vol 46 No 2 (2006): March 2006
Publisher : Indonesian Pediatric Society

Show Abstract | Download Original | Original Source | Check in Google Scholar | DOI: 10.14238/pi46.2.2006.82-6

Abstract

Background Thalassemia is a monogenic disease, yet the clini-cal manifestations (phenotype) are variable although they havethe same genotype. The clear-cut correlation between genotypeand phenotype in β-thalassaemia/HbE patients remains unex-plained. There are several factors that play a role in the severity ofthe clinical manifestations, i.e. two alpha-gene deletion, homozy-gote Xmn1 polymorphism +/+, -+-++, ++-++ haplotype, and hemo-globin Constant Spring.Objective To understand the clinical diversity of patients with HbE/α thalassemia and to determine whether it is possible to predictphenotypic severity from genetic factors.Methods A descriptive study on clinical presentations and hema-tological data of beta-HbE thalassemia patients. DNA analysis wasperformed to detect β-thalassemia mutations and the amelioratingfactors (alpha-globin genes deletions and Xmn1 restriction site poly-morphism at position –158 upstream of the G γ-globin gene) whichwere already known.Results Thirty patients with HbE/β thalassemia (4 to 29 years old)were recruited. IVS1-nt5 (G>C) severe β + mutation was detectedin 20 patients. Eighteen of 20 patients with positive IVS1-nt5 mu-tation group were heterozygous for Xmn1 restriction site polymor-phism and none of the patients was co-inherited with two á-globingene deletion. Almost all patients (19/20) with positive IVS1-nt5mutation group required regular transfusions, yet the mean age atfirst blood transfusion was older in negative IVS1-nt5 mutation groupthan that of positive IVS1-nt5 mutation group (5.7 vs 4 years). Meanhemoglobin before initial transfusion was higher in negative IVS1-nt5 mutation group than that of positive IVS1-nt5 mutation group(5.88 vs 5.39 g/dl). The mean total transfusion per year was lowerin the negative IVS1-nt5 mutation group than that of positive IVS1-nt5 mutation group (190.6 vs 215.1 ml/year).Conclusions Beta-HbE thalassemia patients with identical betathalassemia mutation (IVS1-nt5) show remarkable clinical diver-sity. Neither two alpha-gene deletion, nor the Xmn1- G γ polymor-phism can explain the phenotypic variation. Other amelioratingdeterminants or genetic modifications responsible for the variableclinical severity remain to be explored.