9 research outputs found
Evaluation of Iron Overload Between Age Groups Using Magnetic Resonance Imaging and Its Correlation with Iron Profile in Transfusion-dependent Thalassemia
Get PDFBackground: routine blood transfusion in transfusion-dependent-thalassemia (TDT) causes iron accumulation in various organ. Serum markers of iron overload, serum ferritin and transferrin saturation, are sensitive but not specific. MRI T2-star (T2*) is valuable for detecting iron level in organs. This study aimed to explore the degree of iron overload in various organs, iron deposition difference between children and adults, also its correlation with serum marker of iron overload. Methods: this was a cross-sectional study of TDT patients who had been evaluated by MRI T2* examination in Cipto Mangunkusumo Hospital from 2014 to 2018. Results: a total of 546 subjects was included in this study. The number of subjects between children and adults was almost equal. Most of subjects had normal cardiac iron deposition. The difference of cardiac iron overload between children and adults was significant (p=0.009). Liver evaluation showed that most of subjects had moderate to severe iron overload. This difference between children and adults was significant (p=0.017). Pancreas evaluation showed that either children or adults mostly had mild pancreatic iron overload. Analysis of T2* showed that pancreatic iron deposition progressed with increasing age. Serum ferritin had weak correlation with heart T2* MRI, moderate correlation with pancreas and liver T2* MRI. Relationship between transferrin saturation and T2* MRI was extremely weak. Conclusion: cardiac and hepatic iron deposition between children and adults differ significantly. Liver has the greatest iron overload, followed by pancreas and heart. Iron deposition in liver and pancreas has been started from earlier age. Pancreatic iron deposition rises with increasing age. Serum ferritin and transferrin saturation should not be used solely to predict iron overload in various organs. We suggest that MRI evaluation must be conducted at least once to assess iron deposition in organs
Characteristics of Acute Transfusion Reactions and its related factors in Cipto Mangunkusumo Hospital Jakarta, Indonesia
Get PDFLatar 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, ag
The Importance of Maintaining High Pre-Transfusion Hemoglobin Level in Thalassemia Major Patients
No full textThalassemia patients who do not receive adequate transfusion are at risk of splenomegaly and growth impairment. This study was conducted to evaluate the relation between pre-transfusion hemoglobin (Hb) level with spleen size and body height, also the relation between spleen size and the risk of neutropenia and thrombocytopenia. This was a retrospective study involving 171 non-splenectomized children with thalassemia major. The study was conducted in Dr Cipto Mangunkusumo National Hospital, Jakarta and used data from the past year (2017-2018). Mean pre-transfusion Hb level, neutrophil and thrombocyte count was measured for one year. Mean pre-transfusion Hb level was divided into high (Hb ≥9 g/dL) and low (Hb <9 g/dL). The results showed significant difference of spleen size between subject with high and low pre-transfusion Hb level, but not for the prevalence of stunted. Moderate to massive splenomegaly (spleen size greater than Schuffner 2) increases risk of thrombocytopenia (OR 9.40, p<0.0001), but not for neutropenia (p=0.477). Therefore, it is concluded that maintaining high pre-transfusion Hb level ≥9 g/dL brings many benefits because it prevents the occurrence of complications in children with thalassemia major.  
Glucose and Lipid Profiles in Adolescents with Thalassemia Major and Its Association with Iron Overload in Specific Organs
Get PDFBACKGROUND: Organ damage due to iron toxicity is one factor that increases the risk of getting cardiovascular and metabolic diseases in thalassemia patient. This study aims to determine glucose and lipid profiles in adolescents with thalassemia major and its association with iron overload in pancreas and liver.METHODS: This was a cross sectional study. Subjects were thalassemia major adolescents without any confounding factors that may affect glucose and lipid levels. Blood samples were collected to measure the glucose level, lipid profiles, ferritin level and transferrin saturation. T2-Magnetic Resonance Imaging was used to evaluate the iron overload in organs.RESULTS: From a total of 60 subjects, diabetes mellitus was diagnosed in 1 subject and impaired fasting glucose was diagnosed in 3 subjects. All subjects had high triglycerides/ high density lipoprotein-cholesterol (HDL-C) ratio, 59 subjects (98%) had low HDL-C, 18 subjects (30%) had hypertriglyceridemia, and none had abnormal high level of low density lipoprotein-cholesterol (LDL-C). The majority of subjects had ferritin ≥2,500 ng/mL (70%), mild pancreatic iron overload (56.6%), and moderate hepatic iron overload (43.8%). Degree of hyperferritinemia was not associated with glucose and lipid profiles. Blood glucose profiles were not associated with various degree of pancreatic iron overload. Similar result was also observed between lipid profiles and hepatic iron overload.CONCLUSION: Abnormal glucose and lipid profiles in thalassemia major can be found in adolescence. Normal blood glucose level isn’t necessarily associated with normal pancreatic iron deposition. Hepatic iron overload may worsen dyslipidemia in thalassemia major patients.KEYWORDS: glucose profile, lipid profile, pancreatic iron overload, hepatic iron overload, thalassemia majo
A Rare Case of High Physical Endurance in Transfusion-Dependent Thalassemia Patients with Poor Cardiac Functions
No full textBackground/Objectives: Chronic anemia and iron overload in thalassemia lead to organ failures, including the heart, liver, endocrine glands, and spleen. Comprehensive multidisciplinary management is pivotal in improving patients’ clinical outcomes and well-being. The report aims to present a rare case of improved clinical condition in a transfusion-dependent thalassemia patient. Methods: Medical summaries were collected to compare patients’ conditions at various time frames. Furthermore, the report was composed in chronological order. Results: A 31-year-old male diagnosed with beta-thalassemia major and multiple comorbidities, including diabetes with a history of diabetic ketoacidosis, heart failure with a history of cardiac arrest, hepatomegaly, severe thoracolumbar scoliosis, and depression, exhibited a high physical endurance. The patient managed to maintain a strong treatment adherence and undergo intensive regular multidisciplinary follow-ups. The patient gained cardiac function improvement and metabolic stabilization, leading to completing a 5 k marathon without complication. Conclusions: Intensive management of iron overload through double oral chelation allows organ function improvement. Better mental health attenuates the patient’s overall well-being and is attributed to the ability to gain high physical endurance
Psychological Burden among Pediatric Thalassemia Major Patients in Indonesia: A Review
No full textThalassemia a common hereditary blood disorder resulting in anemia. It is an important public health problem, with a high prevalence in Southeast Asia and Mediterranean countries, and preventable through screening programs. However, due to its chronic nature, permanent physical changes, troublesome complications, and lifelong treatment, pediatric patients with thalassemia major are more prone to mental disorders and cognitive impairment. Internalizing and externalizing problems are higher in pediatric patients with thalassemia. Children with β-thalassemia major exhibit lower IQ scores than healthy children. Neurophysiology and neuroimaging examinations have shown abnormal results in children with thalassemia. Co-morbidity with mental disorders increases the mortality, morbidity, and total healthcare costs of patients with thalassemia. Therefore, routine evaluation of mental health problems is recommended to accommodate the early detection and prompt treatment of mental disorders. A multidisciplinary approach for thalassemia patients and families should be delivered by providing appropriate medical care, psychosocial support, and good transition care to improve survival and well-being, assist good social integration and daily functioning, and cope with the stress of chronic disease
Impact of COVID-19 Pandemic on Pre-Transfusion Hemoglobin Level and Frequency of Transfusion in Transfusion-Dependent Thalassemia Patients in Indonesia
No full textTransfusion-dependent thalassemia is the most severe form of thalassemia; patients require regular blood transfusions to maintain their hemoglobin level. The COVID-19 pandemic has disrupted the routine measures for controlling chronic diseases like thalassemia. This study aims to measure the difference in pre-transfusion hemoglobin levels and the frequency of transfusions before and during pandemic. This retrospective cross-sectional study utilized medical record data of 101 transfusion-dependent thalassemia (TDT) patients treated in Cipto Mangunkusumo Hospital (CMH) from 2019–2021. The dependent variables of this study were pre-transfusion hemoglobin level and transfusion attendance. The pre-pandemic phase was defined as 30 March 2019 to 29 March 2020, whereas the during-pandemic phase was from 30 March 2020 to 29 March 2021. Up to 59.4% of subjects had suboptimal Hb levels of <9.0 g/dL, even before the pandemic, and this increased to 71.3% during the pandemic. The mean pre-transfusion hemoglobin level before the pandemic was 8.71 g/dL, and this decreased to 8.46 g/dL (p value < 0.001). Transfusion attendance before and during the pandemic showed no significant difference (p-value = 0.990). Our study shows poorer control of pre-transfusion Hb levels during the pandemic. This puts patients at higher risk of developing many long-term complications
