Characteristics of Acute Transfusion Reactions and its related factors in Cipto Mangunkusumo Hospital Jakarta, Indonesia

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, ag

Glucose and Lipid Profiles in Adolescents with Thalassemia Major and Its Association with Iron Overload in Specific Organs

BACKGROUND: 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

Liver iron overload and hepatic function in children with thalassemia major

Background Routine blood transfusions and increased intestinal iron absorption lead to iron accumulation in various organs, especially the liver. To date, T2-star magnetic resonance imaging (T2*MRI) is a valuable tool to evaluate iron level in organs. Objective To assess the degree of liver iron overload among children with thalassemia major (TM) and its possible correlations with hepatic function laboratory values. Methods This cross-sectional study was conducted in Cipto Mangunkusumo Hospital. The degree of liver iron overload was evaluated by T2*MRI. Assessments of serum aspartate aminotransferase (AST), alanine aminotransferase (ALT), albumin, and bilirubin levels were done to evaluate liver function. Results A total of 291 TM children were included in this study. The mean age of subjects was 12 years. Most of the subjects were diagnosed as β-thalassemia homozygote (54.6%) and β-thalassemia/HbE (41.2%). Deferiprone (DFP) was the most commonly used iron chelator. Less than 10% of the subjects had normal liver iron deposition. The AST and ALT values increased proportionally with the severity of liver iron overload, with significant, moderately negative correlation coefficients (r=-0.388 and -0.434, respectively). However, albumin level decreased proportionally with the severity of liver iron overload, with a significant, moderately positive correlation coefficient (r=0.323). Liver T2* MRI had no significant correlations with direct, indirect, and ratio of direct/total bilirubin levels. Conclusion Most of the children with TM have mild to severe liver iron overload. Liver T2* MRI had significant, moderate correlations with AST, ALT, and albumin values. Bilirubin level has no correlation with T2* MRI. Our findings suggest that monitoring of AST, ALT, and albumin levels is important because they may reflect the severity of liver iron overload. However, they should not be used as the only predictors of iron overload

Hemostatic abnormalities in children with thalassemia major and liver iron overload

Background Thalassemia major (TM) patients are susceptible to liver dysfunction due to iron deposition. Pediatric TM patients often present with bleeding. Blood loss necessitates transfusions, leading to increased iron absorption from the gut. Objective To study hemostatic abnormalities in children with TM and iron deposition in the liver. Methods This cross-sectional study involved 190 non-splenectomized children with TM. Liver iron deposition was evaluated using T2* MRI. Prothrombin time (PT), activated partial thromboplastin time (aPTT), and platelet counts were assessed from blood specimens. Results Most subjects were diagnosed with β-thalassemia and β-thalassemia/HbE. The majority of subjects were on deferiprone (DFP) treatment. Approximately 89.5% of subjects had liver iron overload. Prolongation of PT and aPTT, as well as thrombocytopenia were observed in 60%, 27.9%, and 19.5% of subjects, respectively. Prolonged aPTT and thrombocytopenia were observed three times more frequently in subjects with moderate - severe liver iron overload than in subjects with normal - mild liver iron overload (P=0.04 and 0.001, respectively). Conclusion Most TM subjects have liver iron overload ranging from mild to severe. Prothrombin time and prolongation, as well as aPTT prolongation, and thrombocytopenia are easily found in TM children. There were significantly more moderate - severe liver iron deposition patients with aPTT prolongation and thrombocytopenia than normal – mild patients with these conditions. Hence, we suggest that pediatric TM patients undergo liver iron deposition evaluations and use iron chelators in an optimal manner, in order to limit the risk of bleeding

The Importance of Maintaining High Pre-Transfusion Hemoglobin Level in Thalassemia Major Patients

Thalassemia 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. &nbsp

Evaluation of Iron Overload Between Age Groups Using Magnetic Resonance Imaging and Its Correlation with Iron Profile in Transfusion-dependent Thalassemia

Background: 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

Gestational age, birth weight, and blood culture microbial patterns in late-onset neonatal sepsis

Background The three main  causes of neonatal deaths in Indonesia according to the WHO are sepsis, prematurity, and asphyxia. A suboptimal hospital environment increases the risk of late-onset neonatal sepsis (LONS), which in turn can prolong hospital stays. Objective To assess for possible associations  of bacterial patterns in neonates with LONS, prematurity, and/or low birth weight. Methods Medical record data of 1,706 hospitalized neonates who were treated for sepsis or other disease in the Neonatal Unit, Dr. Cipto Mangunkusumo Hospital (CMH), Jakarta in 2020 were analyzed retrospectively. A total of 262 neonates had proven LONS. We assessed for possible risk factors such as gestational age, birth weight, and cultured blood microbes. Results Out of a total of 1,706 neonates admitted to the neonatal unit, the incidence of proven LONS was 15.4%. LONS was more prevalent (58.4%) in preterm than in full-term (41.6%) neonates. The majority (67.6%) of subjects with proven LONS were neonates with low birth weight (LBW) (<2,500 grams), and the largest percentage of them (35.1%) was in the 1,500-2,500-gram group. Gram negative bacteria emerged as the predominant pathogens of LONS patients in our hospital; the most common were Klebsiella pneumonia, Acinetobacter spp., Escherichia coli, Enterobacter spp., and Pseudomonas aeruginosa. Conclusion The proportion of LONS among LBW and preterm neonates is significantly higher compared to normal birth weight and  neonates. In our unit, LONS was mostly caused by Gram-negative bacteria. The antibiotic susceptibility of the various pathogens causing LONS in CMH should be tested and compared to the current empirical antibiotic guidelines used in CMH