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Query: UMLS:C0002895 (sickle cell disease)
 11,747 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

In 1,814 patients with sickle cell disease who had been transfused, the overall rate of alloimmunization to erythrocyte antigens was 18.6%. The rate of alloimmunization in this group appears to be an explicit function of the number of transfusions received because it increases exponentially with increasing numbers of transfusions. Alloimmunization usually occurred with less than 15 transfusions, although the rate of alloimmunization continued to increase when more transfusions were given. The rate of alloimmunization was less in patients with hemoglobin SC disease and sickle-beta+ thalassemia because these patients had received fewer transfusions. Children less than 10 years old had a slightly lower rate of alloimmunization than patients in other age groups even after correction for the number of transfusions given. Women were more frequently alloimmunized than men; this was largely due to the fact that women received more transfusions than men, but in the age group 16 to 20 years the increase may have been due in part to alloimmunization owing to pregnancy. Forty-five percent of those alloimmunized made antibodies of only one specificity; 17% made four or more antibodies reacting with different antigens. Antibodies to the C and E antigens of the Rh group, the Kell antigen, and the Lewis antigens were most commonly made. These findings may be important in formulating a rational transfusion policy in sickle cell disease.
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PMID:Transfusion and alloimmunization in sickle cell disease. The Cooperative Study of Sickle Cell Disease. 220 18

Alloimmunization to red cell antigens contributes to morbidity in transfused patients. It has been recommended that blood for sickle cell patients need not be matched for antigens other than ABO and Rh(D), as there is no greater incidence of antibody production than in other multitransfused patient populations. Post transfusion alloimmunization was studied in a group of 34 sickle cell disease patients attending a U.K. haemoglobinopathy clinic. Red cell antibodies were formed in 17.6% of the transfused patients and Rhesus and Kell antibodies accounted for 66% of this total. In order to reduce alloimmunization, a policy of performing extended red cell phenotyping on the patients, and providing blood matched for Kell, and in certain circumstances the Rhesus antigens other than Rh(D), is recommended.
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PMID:Red cell alloimmunization in sickle cell disease. 308 6

The present study was carried out to determine the evidence of alloimmunization against red blood cells in 364 patients transfused in our center over a period of 4 years (1990-1993). Among these patients, 127 were thalassemic and 182 had sickle cell disease (SCD). In 55 control patients, who received blood matched for the ABO, Rhesus and Kell antigen systems from the outset of transfusion, no immunization was detected. However, in the study group, who initially received blood matched only for ABH and Rh D antigens, the frequency of alloimmunization was 7.76% (24/309). Only one antibody was detected in 15 patients (62.5%) and two or more in 9 patients (37.5%). Alloimmunization concerned the Rhesus system in 58.82% of cases and the Kell system in 26.47%, while the frequency of immunization was significantly lower in patients of less than 5 years as compared to those in the age range 5-10 years (p < 0.001).
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PMID:Red cell alloantibodies in patients with haemoglobinopathies. 789 31

Alloimmunization following multiple transfusions, a frequently observed complication in sickle cell anemia patients, may make subsequent transfusion attempts extremely hazardous. The case of a young woman with sickle-cell anemia is reported; she was hospitalized for the treatment of extensive invalidating leg ulcers. The transfusion program that was initiated as a prerequisite to skin allografts had to be stopped, due to rapid occurrence of multiple alloimmunization. Broad spectrum alloantibodies precluded further selection of compatible blood units. In an attempt to get round this impossibility, a treatment with Hydroxyurea was initiated in order to boost synthesis of F-hemoglobin. After one year under this treatment, the patient's clinical status has clearly improved without requiring any new blood transfusion.
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PMID:[A sickle cell homozygote with transfusion deadlock. Favorable outcome with hydroxyurea treatment]. 811 73

Management of transfusion therapy in sickle cell disease patients with acute complications is often made difficult because of confusing indications, a variety of methods, disparate goals, and varying needs for maintenance transfusion. In priapism, acute chest syndrome, many major surgical procedures, toxemia of pregnancy, and cerebrovascular accidents, the target hemoglobin A level should be made as close to 100% as possible by mechanized red blood cell exchange. If mechanized exchange is unavailable, manual exchange should be instituted. Hemoglobin A should be maintained at greater than 60% to 70% by periodic simple transfusion until patients are fully recovered. Stroke patients should undergo maintenance transfusions for at least 3 years and perhaps 5 to 12 years. Physicians and patients should be aware of the transfusion-related risks of hepatitis and HIV infection. Alloimmunization and iron overload should be minimized in patients requiring frequent transfusions and chelation therapy should be utilized for iron overload.
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PMID:Transfusion therapy in sickle cell disease patients: methods and acute indications. 812 Apr 39

Blood transfusion in patients with sickle cell disease (SCD) is limited by the development of alloantibodies to erythrocytes. In the present study, the frequency and risk factors for alloimmunization were determined. Transfusion records and medical charts of 828 SCD patients who had been transfused and followed at the Belo Horizonte Blood Center, Belo Horizonte, MG, Brazil, were retrospectively reviewed. Alloimmunization frequency was 9.9% (95% CI: 7.9 to 11.9%) and 125 alloantibodies were detected, 79% of which belonged to the Rhesus and Kell systems. Female patients developed alloimmunization more frequently (P = 0.03). The median age of the alloimmunized group was 23.3 years, compared to 14.6 years for the non-alloimmunized group (P < 0.0001). Multivariate analyses were applied to the data for 608 hemoglobin (Hb) SS or SC patients whose number of transfusions was recorded accurately. Number of transfusions (P = 0.00006), older age (P = 0.056) and Hb SC (P = 0.02) showed independent statistical associations with alloimmunization. Hb SC patients older than 14 years faced a 2.8-fold higher (95% CI: 1.3 to 6.0) risk of alloimmunization than Hb SS patients. Female Hb SC patients had the highest risk of developing alloantibodies. In patients younger than 14 years, only the number of transfusions was significant. We conclude that an increased risk of alloimmunization was associated with older patients with Hb SC, specially females, even after adjustments were made for the number of transfusions received, the most significant variable.
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PMID:Risk factors for alloimmunization by patients with sickle cell disease. 1591 48

Thirteen adult patients aged 22-63 (median 30) years with sickle cell disease (SCD) were enrolled in a regular erythrocytapheresis (ECP) programme at a single institution between December 1998 and November 2008. The indications for enrolment were recurrent painful crises (PC), acute chest syndrome (ACS), silent cortical ischaemia, pulmonary hypertension, multi-organ crises and pregnancy. Endpoints retrospectively evaluated included the incidence of SCD-related acute events requiring hospitalization following and prior to regular ECP, the development of new and progression of pre-existing related end-organ damage, the effectiveness in reducing HbS levels acutely and prior to the next exchange and the transfusion-related complications. Sixteen acute sickle-related events occurred in five patients in 846 months of patient follow-up. In all patients with reliable data available pre-ECP, the frequency of such events was reduced following commencing regular ECP. No patient experienced stroke, multi-organ crises or developed new and/or progression of end-organ dysfunction. Regular ECP reduced HbS levels to the target of <30% immediately post-exchange. Alloimmunization rates were comparable to the literature and ECP was effective in preventing progressive iron overload. Regular ECP was demonstrated to be an effective, well-tolerated therapy for both acute and chronic complications of SCD in adults.
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PMID:The impact of a regular erythrocytapheresis programme on the acute and chronic complications of sickle cell disease in adults. 2151 12

Sickle cell disease (SCD) is an important public health issue in Bahia, Brazil. Erythrocyte transfusions may reduce morbidity of SCD, however, they are associated with numerous risks. Among other risk categories, alloimmunization to red cell antigens may result from transfusions. The aim of this study was to compare the clinical profile of transfused adult SCD patients with and without alloantibodies. The study included 108 patients (105 homozygous SS and three with hemoglobinopathy SC), followed in the Outpatient Unit of the Hematology and Hemotherapy Center of Bahia. A retrospective review of clinical records of adult SCD patients who received at least three red blood cell transfusions from 2004 to 2007 was performed. Transfusion units were phenotypically matched for ABH-D and C,c,E,e, and K antigens. Alloimmunization developed in 56 patients (53 SS and three SC). The most prevalent alloantibodies were anti-E, anti-K, and anti-C (39.3%, 21.4%, and 16.1%, respectively). Age, sex and positive antiglobulin test displayed statistically significant differences. Prevalence of clinical complications such as leg ulcers, stroke, and others did not show differences between groups. In conclusion, alloimmunization did not significantly modify the clinical outcomes of SCD patients from Bahia, Brazil.
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PMID:Alloimmunization and clinical profile of sickle cell disease patients from Salvador-Brazil. 2050 98

Transfusion remains the main treatment of sickle cell disease patients. Red cell alloimmunization is frequent because of the antigen disparities between patients of African descent and donors of European ancestry. Alloimmunization is associated with severe hemolytic transfusion reaction, autoantibody formation, and difficulties in the management of transfusion compatibility. Beside common antigens, a number of different RH variant antigens found in individuals of African descent can be involved in alloimmunization. If some variants, such as Hr(S) negative antigens, are known to prone significant alloantibodies and delayed hemolytic transfusion reactions, it is not clear whether all the described variants represent a clinical risk for sickle cell disease patients. The knowledge of the clinical relevance of RH variants is a real issue. An abundance of molecular tools are developed to detect variants, but they do not distinguish those likely to prone immunization from those that are unlikely to prone immunization and delayed hemolytic transfusion reactions. A strategy of prevention, which generally requires rare red blood cells, cannot be implemented without this fundamental information. In this review, we discuss the relevance of RH variants in sickle cell disease, based on the published data and on our experience in transfusion of these patients.
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PMID:Relevance of RH variants in transfusion of sickle cell patients. 2202 28

Sickle cell disease is a hereditary pathology of the haemoglobin which affects only individuals from African ancestry. The frequency of the disease increases in France. Transfusion remains a major treatment of this disease. Depending of the indication, transfusion can be a simple transfusion or an exchange transfusion. In this last case, exchange can be performed manually or automatically. The transfusion protocols have to be adapted to the polytransfused status of these patients, but also to the high incidence of alloimmunisation against red blood cells. Alloimmunisation is a consequence of the polymorphism of blood groups between sickle cell disease patients and donors of European ancestry. Axes to optimize transfusion safety in these patients have to be developed. But the first step relies on the promotion of blood donation within individuals of African ancestry.
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PMID:[Transfusion and sickle cell disease]. 2359 85


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