Erythrocyte autoantibodies in paediatric patients with sickle cell disease receiving transfusion therapy: frequency, characteristics and significance

Castellino, Sharon M.; Combs, Martha Rae; Zimmerman, Sherri A.; Issitt, Peter D.; Ware, Russell E.

doi:10.1046/j.1365-2141.1999.01127.x

Cited by 114 publications

(89 citation statements)

References 48 publications

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“…It was also suggested that alloantibody binding to the RBCs could lead to conformational changes of the antigenic epitope that ultimately stimulates production of autoantibodies [42]. It is possible that certain people are genetic responders who have an increased tendency to develop RBC autoantibodies and the tendency toward autoantibody formation could reflect an overall dysfunction of the immune system [43].In study by Ahmed there was a significant association of splenectomy with alloimmunization and auto antibodies (P = 0.03, 0.001 respectively).Wiener et al [44], demonstrated significant elevations in RBC bound IgG in thalassaemia patients which was more abundant in splenectomized than nonsplenectomized subjects. They found that the absence of a spleen may further enhance the immune response to the infused foreign antigens which are not effectively filtered.…”

Section: Other Factorsmentioning

confidence: 99%

Red Blood Cell Antibodies in Thalassemia Patients in Northern India: Risk Factors and Literature Review

Elhence

Solanki

Verma

2014

Indian J Hematol Blood Transfus

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Study of the factors responsible for red cell alloimmunization can help in adopting appropriate strategy to minimize alloimmunization. However data for thalassemia patients from our region is limited. Therefore, a study was conducted to find out the frequency and the factors associated with red cell allo and autoimmunization in thalassemia patients at our center so as to enable us to take appropriate action to reduce alloimmunization. Clinical, demographic, allo and autoantibody and transfusion records of 280 thalassemia patients at our hospital were studied. Patients with and without alloantibodies were compared to find significant differences for age, gender, race, age at start of regular transfusions and splenectomy. Red cell antigen frequencies in thalassemia patients and published antigen frequencies in blood donors from the same center were compared to look antigen differences as a risk factor for alloimmunization. Twenty four thalassemia patients (8.6 %) developed 28 clinically significant alloantibodies. 18 (65 %) of the alloantibodies were of Rh system. The three most common antibodies detected was anti E (11, 39.3 %) followed by anti K (6, 21.4 %) and anti c (10.8 %). Five (1.8 %) of the 280 patients developed autoantibodies. Patient age was found to be significantly higher in alloimmunized patients than in non alloimmunized patients. Red cell antigen frequencies between blood donor and recipient populations were found to be homogenous for most of the relevant RBC antigens. The frequency of red cell alloimmunization in thalassemia patients from our center is moderate. In this setting of red cell phenotype concordant donor-recipient population requirement of extended phenotype matched transfusions may not be cost effective.

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Section: Other Factorsmentioning

confidence: 99%

Red Blood Cell Antibodies in Thalassemia Patients in Northern India: Risk Factors and Literature Review

Elhence

Solanki

Verma

2014

Indian J Hematol Blood Transfus

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“…59 In the STOP I trial, matching for the C, E, and K antigens decreased the rate of alloimmunization by a factor of 6 per unit of exposure. 60 Although extensive blood type matching can be challenging, blood transfusions containing antigens to which the donor is alloimmunized not only increases the risk of hemolytic reactions but also shortens the half-life of the infused RBCs 61 ; consequently, additional transfusions are required to maintain a low HbS, which further increases the risk of iron overload, infection, and alloimmunization.…”

Section: Extended Blood Typingmentioning

confidence: 99%

Sickle cell disease and stroke

Verduzco

Nathan

2009

Blood

163

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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“…9 Patients presenting at the age 1 or 2 years of life were transfusion dependent for life. 10 Majority of our patients started on transfusions between the ages of 3month to 1 year old. One patient started transfusion at the age of 1month, while one patient started transfusion at 3years old.…”

Section: Efficacy Of Transfusion Regimementioning

confidence: 99%