Frequency of Antibodies to Various Antigenic
Determinants in Polytransfused Patients with
Homozygous Thalassaemia in Greece

Economidou, J.; Constantoulakis, M.; Augoustaki, Olga; Adinolfi, M.

doi:10.1159/000466635

Cited by 21 publications

(17 citation statements)

References 10 publications

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“…In the Cooperative Study of Sickle Cell Disease (CSSCD) 47 (NCT00005277), the overall rate of alloimmunization in SCD patients was 18.6%, and this prevalence was directly related to the number of blood transfusions received. This rate is higher than that observed in patients with thalassemia, [48][49][50][51] probably because in SCD the donor and recipient pools differ racially 34 and because transfusions in thalassemia begin at an early age and are maintained. This contributes to immune paralysis.…”

Section: Extended Blood Typingmentioning

confidence: 71%

Sickle cell disease and stroke

Verduzco

Nathan

2009

Blood

167

121

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Twenty-four percent of sickle cell disease (SCD) patients have a stroke by the age of 45 years. Blood transfusions decrease stroke risk in patients deemed high risk by transcranial Doppler. However, transcranial Doppler has poor specificity, and transfusions are limited by alloimmunization and iron overload. Transfusion withdrawal may be associated with an increased rebound stroke risk. Extended blood typing decreases alloimmunization in SCD but is not universally adopted. Transfusions for thalassemia begun in early childhood are associated with lower rates of alloimmunization than are seen in SCD, suggesting immune tolerance. Optimal oxygen transport efficiency occurs at a relatively low hematocrit for SCD patients because of hyperviscosity. Consequently, exchange rather than simple transfusions are more effective in improving oxygen transport efficiency, but the former are technically more demanding and require more blood units. IntroductionSickle cell disease (SCD) is a protean disorder caused by elevations of intraerythrocyte and total blood viscosity. Hypoxiainduced gelation of hemoglobin S (HbS) deforms the erythrocyte and its membrane and causes massive cation loss as well as increased erythrocyte surface expression of adhesion molecule receptors. The concomitant lack of deformability and enhanced stickiness lead to obstructive adhesion of sickle cells to each other and to vascular endothelium. 1 The result is ischemia reperfusion injury and endothelial cell damage with leakage of von Willebrand factor, platelet clumping, cytokine release, and attraction of granulocytes, macrophages, T cells, and invariant natural killer T (iNKT) cells to areas that become both infarcted and inflamed. 2,3 The obstruction and inflammation in turn cause further hypoxia and acidosis and, consequently, further sickling, the so-called vicious cycle of SCD.Of the many severe consequences of SCD, acute stroke and chronic cerebral ischemia are among the most disabling. 4 The fragile cerebral vessels of young children are particularly vulnerable: crippling strokes may blight a child's life even before the age of 2 years. The terrible consequences of SCD-induced stroke have prompted multiple approaches to prevention of this pernicious complication.Stroke in SCD was initially noted in 1923, 5 13 years after the first description of the disease. 6 A landmark angiographic case study in 1972 demonstrated the particular vulnerability of the internal carotid artery and circle of Willis 7 with consequent formation of fragile collaterals. This can lead to Moyamoya syndrome, a characteristic finding on cerebral angiograms consisting of multiple small collateral vessels around the circle of Willis that give a "puff of smoke" appearance.In this review, we offer a brief discussion of the natural history and prevention of stroke in SCD. We first discuss secondary prevention studies because they have led to landmark trials of primary prevention strategies. The review then highlights the potential pitfalls of nonspecific imaging modalities to sc...

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Section: Extended Blood Typingmentioning

confidence: 71%

Sickle cell disease and stroke

Verduzco

Nathan

2009

Blood

167

121

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“…Various centers all around the world have reported different frequencies of alloimmunization. The frequency of red cell alloimmunization ranging from 3.1 to 30 % [1][2][3][4][5][6][7][8][9][10][11][12][13][14][15][16][17][18]. The some of these reports have a high rate of alloimmunization [2,6,8,15] but majority of centers have reported low rate of alloimmunization [3, 12, 13, 16-18, 20, 21].…”

Section: Discussionmentioning

confidence: 99%

“…The most frequent detected antibodies are directed against Rhesus (Rh), Kell (K), Duffy (Fy), Kidd (Jk) system antigens, in order of frequency [3]. The reported incidence of alloimmunization in multiply transfused thalassemics patients was 3-30 % [1][2][3][4][5][6][7][8][9][10][11][12][13][14][15][16][17][18].…”

Section: Introductionmentioning

confidence: 99%

Detection and Identification of Red Cell Alloantibodies in Multiply Transfused Thalassemia Major Patients: A Prospective Study

Jain

Choudhury

Chudgar³

et al. 2013

Indian J Hematol Blood Transfus

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Life long red blood transfusion remains the main treatment for b thalassemia major patients. The development of alloantibodies complicates transfusion therapy in thalassemia patients. Alloimmunization to red cell antigens is one of the most important immunological transfusion reaction and causes delayed type of transfusion reaction. A prospective study was conducted from January 2007 to January 2010. This was a cohorts of 115 patients were selected from regular transfusion group and they were followed for two and half year. They were followed up for the effect of transfusion during study period. There was a decline in patient number from 115 to 96 due to mortality and transfer of patient. A total of 96 multiply transfused thalassemia patients were prospectively included in this study and three consecutive samples collected after every 6 months and investigated for the development of alloantibody to red cell antigens. Tests for antibody screening and identification were performed on preserved sample to investigate prevalence and development of red cell alloimmunization by standardized laboratory techniques by same person at Prathama Blood Centre. A total of 96 patients were included in the study. 63 patients were males and 33 females. A total of five single alloantibodies were formed in five patients out of them four (80 %) belonged to Kell blood group system and one (20 %) from Rh system. It was observed that two (1.92 %) of new thalassemia patients developed red cell alloantibodies during study period. Red cell alloimmunization should be kept in mind in the patients receiving multiple transfusions. In present study, alloimmunization rate was 5.21 %. Mean transfusion duration in these patients was 23.90 days, probably due to presence of alloantibody. RBC alloantibody detection on regular interval and corresponding antigen negative blood transfusion is strongly recommended in transfusion dependent thalassemia patients.

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“…However, alloimmunization to minor RBC antigens is frequent in chronically transfused individuals, such as those with severe thalassemias and sickle cell anemia (27,28). In such patients, 20-30% will ultimately show evidence of alloimmunization against minor blood group antigens making subsequent transfusions more problematic (2,29). Finally, a large (but unknown) measure of morbidity and mortality may also be ascribed to the lack of transfusion.…”

Section: Discussionmentioning

confidence: 99%

Chemical camouflage of antigenic determinants: Stealth erythrocytes

Scott

Murad

Koumpouras

et al. 1997

Proc. Natl. Acad. Sci. U.S.A.

208

215

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In a number of clinical circumstances it would be desirable to artificially conceal cellular antigenic determinants to permit survival of heterologous donor cells. A case in point is the problem encountered in transfusions of patients with rare blood types or chronically transfused patients who become allosensitized to minor blood group determinants. We have tested the possibility that chemical modification of the red blood cell (RBC) membrane might serve to occlude antigenic determinants, thereby minimizing transfusion reactions. To this end, we have covalently bound methoxy(polyethylene glycol) (mPEG) to the surface of mammalian RBC via cyanuric chloride coupling. Human RBC treated with this technique lose ABO blood group reactivity as assessed by solution-phase antisera agglutination. In accord with this, we also find a profound decrease in anti-blood group antibody binding. Furthermore, whereas human monocytes avidly phagocytose untreated sheep RBC, mPEG-derivatized sheep RBC are ineffectively phagocytosed. Surprisingly, human and mouse RBC appear unaffected by this covalent modification of the cell membrane. Thus, mPEG-treated RBC are morphologically normal, have normal osmotic fragility, and mPEG-derivatized murine RBC have normal in vivo survival, even following repeated infusions. Finally, in preliminary experiments, mPEG-modified sheep RBC intraperitoneally transfused into mice show significantly improved (up to 360-fold) survival when compared with untreated sheep RBC. We speculate that similar chemical camouf lage of intact cells may have significant clinical applications in both transfusion (e.g., allosensitization and autoimmune hemolytic disease) and transplantation (e.g., endothelial cells and pancreatic ␤ cells) medicine.The transfusion of red blood cells (RBC) is the most common, and best tolerated, form of tissue transplantation. Indeed, in 1993, over 14 million units of blood were donated for transfusion in the United States alone (1). In most transfusions, simple blood typing (ABO͞Rh-D) is sufficient to identify appropriate donors. Occasionally, however, appropriate donors for patients with rare blood types cannot quickly be found; a situation that may become life-threatening. More often, problems are encountered in individuals who receive chronic transfusions, such as patients with sickle cell anemia and thalassemia. In such patients, alloimmunization against minor RBC antigens can make it nearly impossible to identify appropriate blood donors (2-4). Previous studies in which purified proteins were covalently modified with poly(ethylene glycol) (PEG) provided a possible solution to this problem. PEG-modified proteins have been shown to have increased in vivo survival and to be nonimmunogenic, even with repeated infusions (5, 6). We therefore explored the hypothesis that the covalent binding of PEG to intact RBC might mask RBC surface antigens and thereby permit the survival of heterologous or even xenogeneic RBC.To experimentally test this hypothesis, human, mouse, and sheep RBC were...

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Frequency of Antibodies to Various Antigenic Determinants in Polytransfused Patients with Homozygous Thalassaemia in Greece

Cited by 21 publications

References 10 publications

Sickle cell disease and stroke

Sickle cell disease and stroke

Detection and Identification of Red Cell Alloantibodies in Multiply Transfused Thalassemia Major Patients: A Prospective Study

Chemical camouflage of antigenic determinants: Stealth erythrocytes

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