Infections in Thalassemia and Hemoglobinopathies

Ricerca, Bianca Maria; Girolamo, Arturo Di; Rund, Deborah

doi:10.4084/mjhid.2009.028

Cited by 51 publications

(63 citation statements)

References 116 publications

(117 reference statements)

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“…Interestingly, patients with transfusional iron overload, as well as those with hereditary forms of hemochromatosis, may have circulating non-transferrin-bound iron levels 25,26 similar to those measured in our healthy volunteers at 4 hours after transfusion with older RBCs and are known to be at an increased risk for acute and chronic infections with specific pathogens. [27][28][29] In addition, oral iron supplements are associated with transient increases in non-transferrin-bound iron, 19,30 and routine supplementation with iron and folic acid in children in a malaria-endemic region increased the risk of severe illness and death. 31,32 An incidental finding from this study is the extent of variability in hemoglobin levels measured soon after transfusion ( Figure 1B-C), with 6 of 14 volunteers exhibiting either a decrease or no increase in hemoglobin at 4 hours after transfusion of fresh autologous RBCs.…”

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confidence: 99%

Transfusion of human volunteers with older, stored red blood cells produces extravascular hemolysis and circulating non–transferrin-bound iron

Hod¹,

Brittenham

Billote

et al. 2011

Blood

256

286

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Transfusions of RBCs stored for longer durations are associated with adverse effects in hospitalized patients. We prospectively studied 14 healthy human volunteers who donated standard leukoreduced, double RBC units. One unit was autologously transfused "fresh" (3-7 days of storage), and the other "older" unit was transfused after 40 to 42 days of storage. Of the routine laboratory parameters measured at defined times surrounding transfusion, significant differences between fresh and older transfusions were only observed in iron parameters and markers of extravascular hemolysis. IntroductionThe safety of transfusing RBCs after longer durations of refrigerated storage was recently identified as "the most critical issue facing transfusion medicine." 1 page 667 Concern was heightened when a large observational study of cardiac surgery patients found an increased risk of postoperative complications and reduced survival in those who received RBCs stored for more than 14 days. 2 Although still controversial, adverse clinical consequences have since been reported in most, [3][4][5] although not all, 6,7 epidemiologic studies of transfusions of RBCs stored for longer durations, but still within Food and Drug Administration (FDA) guidelines. The association between the duration of RBC storage and increased rates of serious infections, sepsis, and mortality is particularly strong in trauma patients. [7][8][9][10][11] Definitive determination of the potential risks associated with transfusion of RBCs stored for longer durations has been elusive, in part because the mechanisms responsible have not yet been identified.More than 14 million RBC units are transfused in the United States each year, with a mean storage interval of 18 days before transfusion. 12 During storage, RBCs undergo cumulative biochemical and biomechanical changes (the "storage lesion") that reduce their survival in vivo after transfusion. 13,14 In mouse models, 15 transfusion of RBCs stored for longer durations was followed by brisk extravascular clearance of a subpopulation of these cells, which were damaged during storage and removed by macrophages in the spleen and liver of recipient mice. The iron liberated by phagocytic digestion of these RBCs rapidly entered the systemic circulation in amounts that exceeded the transport capacity of plasma transferrin, the physiologic iron-binding protein; in this way, circulating non-transferrin-bound iron appeared and promoted the proliferation of pathogenic bacteria both in vitro 15 and in vivo. 16 We hypothesized that the infectious complications observed in human patients after transfusion of RBCs stored for longer durations were, at least in part, the result of the production of circulating non-transferrin-bound iron. Therefore, we prospectively examined healthy human volunteers to determine (1) if transfusion of autologous RBCs stored for longer durations was followed by the appearance of circulating non-transferrin-bound iron in vivo, and (2) if this increased circulating non-transferrinbound iron was assoc...

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confidence: 99%

Transfusion of human volunteers with older, stored red blood cells produces extravascular hemolysis and circulating non–transferrin-bound iron

Hod¹,

Brittenham

Billote

et al. 2011

Blood

256

286

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“…Eritropoietik inefektif dan hemolisis menyebabkan monosit/makrofag menjadi hiperplasia dan hiperaktif memfagositosis prekursor eritrosit yang rusak dan juga eritrosit matur. 9 Anemia yang berat pada pasien talasemia, juga merupakan faktor risiko terjadinya infeksi. 17 Pemberian transfusi darah berulang berhubungan dengan stimulasi aloantigenik terus menerus dan juga berhubungan dengan hemolisis autoimun.…”

Section: Respons Imun Pada Talasemiaunclassified

“…18 Limpa berperan penting dalam respons imun karena limpa merupakan sumber penting limfosit imunokompeten. 9 Limpa dan kelenjar getah bening merupakan tempat sel dendritik mempresentasikan antigen yang ditangkap di bagian lain tubuh ke sel T yang memacunya untuk proliferasi dan diferensiasi limfosit. 22 Secara umum, limpa berfungsi untuk memfiltrasi antigen dan mikroorganisme dan juga berfungsi dalam proses sintesis antibodi dan komplemen.…”

Section: Seng Dan Respons Imununclassified

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Seng dan Respons Imun pada Talasemia

Sari

2016

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Infeksi merupakan morbiditas dan mortalitas yang penting pada pasien talasemia. Mekanisme terjadinya infeksi yang berperan adalah gangguan respons imun akibat patofisiologi penyakit talasemia, kelebihan besi, splenektomi dan defisiensi seng. Seng merupakan zat penting pada respons imun yang dihubungkan dengan akivitas timulin. Kadar feritin yang tinggi juga memengaruhi kadar seng pada pasien talasemia. Suplementasi seng memperbaiki kadar seng yang berakibat pada perbaikan stres oksidatif dan respons imun pada pasien talasemia. Sari Pediatri 2016;18(2):157-63Kata kunci: seng, respons imun, talasemia Zinc and Immune Responses in ThalassemiaTeny Tjitra Sari Infection is one of important morbidity and mortality in thalassemia patients. The mechanism infections are impaired immune response due to pathophysiology of thalassemia, iron overload, splenectomy, and zinc deficiency. Zinc plays important role in immune response associated with thymulin activity. High ferritin also influenced zinc level in thalassemia patients. Zinc supplementation results in increasing zinc level, improved oxidative stress and immune response in thalassemia patients. Sari Pediatri 2016;18(2):157-63

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“…Severe anaemia, iron overload, splenectomy, and a range of other immune abnormalities can be considered as some of the risk factors causing infections in these patients [3,4]. Infections are considered as the second most common cause of morbidity in thalassemia patients [4].…”

Section: Alpha (α) or Beta (β) Thalassemia Traitmentioning

confidence: 99%