{"title":"亚历山大大学儿童医院输血依赖性β-地中海贫血患者的红细胞异体免疫","authors":"Nehad Hassanein, Bothaina El-Domiaty, Rovaida El-Fawal, Nevien Mikhail","doi":"10.4103/ajop.ajop_5_22","DOIUrl":null,"url":null,"abstract":"Background In Egypt, it has been established that the most frequently inherited disorder of hemoglobin is β-thalassemia. Management of the severe forms of this disorder consists chiefly of red cell transfusion. The concerned patients receive these transfusions on a regular basis. Therefore, the formation of antired cell alloantibodies and autoantibodies was inevitable. This dilemma is addressed in this study that aims to determine the prevalence of red cell alloimmunization among transfusion-dependent ß-thalassemia children that receive frequent care in the Hematology Clinic of Alexandria University Children’s Hospital. Patients and methods This study screened 100 transfusion-dependent patients in total, all of them were 3–16 years old. Transfusion and clinical records of the latter were reviewed to assess the diagnosis of β-thalassemia, the age of the first presentation, the age at which transfusion therapy was started, transfusion interval, and the number of red cell units given. Alloantibody detection was then performed. Results In the present study, 11 (11%) out of the total 100 studied patients were alloimmunized. The total number of alloantibodies identified in these patients was 25. Two patients (18%) had only one alloantibody, six patients (54.5%) had two alloantibodies, one patient (9%) had three alloantibodies, and two patients (18%) had more than three alloantibodies. The most prevalent antibodies belonged to Kell and Rh blood-group systems (seven out of 25 each). Conclusions When it comes to thalassemia patients dependent on blood transfusion, red blood cell alloimmunization is a difficult complication. Kell and RH blood-group-system antibodies are the predominant antibodies. Formation of alloantibodies depends mainly on the nature of red cell units received and donor and recipient factors. These can explain the difference in alloimmunization rates in different reports. Avoidance of alloimmunization would decrease the cost of treatment on the long run and improve the patient quality of life.","PeriodicalId":7866,"journal":{"name":"Alexandria Journal of Pediatrics","volume":"54 1","pages":"26 - 32"},"PeriodicalIF":0.0000,"publicationDate":"2022-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"Red cell alloimmunization in transfusion-dependent β-thalassemia patients attending Alexandria University Children Hospital\",\"authors\":\"Nehad Hassanein, Bothaina El-Domiaty, Rovaida El-Fawal, Nevien Mikhail\",\"doi\":\"10.4103/ajop.ajop_5_22\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"Background In Egypt, it has been established that the most frequently inherited disorder of hemoglobin is β-thalassemia. Management of the severe forms of this disorder consists chiefly of red cell transfusion. The concerned patients receive these transfusions on a regular basis. Therefore, the formation of antired cell alloantibodies and autoantibodies was inevitable. This dilemma is addressed in this study that aims to determine the prevalence of red cell alloimmunization among transfusion-dependent ß-thalassemia children that receive frequent care in the Hematology Clinic of Alexandria University Children’s Hospital. Patients and methods This study screened 100 transfusion-dependent patients in total, all of them were 3–16 years old. Transfusion and clinical records of the latter were reviewed to assess the diagnosis of β-thalassemia, the age of the first presentation, the age at which transfusion therapy was started, transfusion interval, and the number of red cell units given. Alloantibody detection was then performed. Results In the present study, 11 (11%) out of the total 100 studied patients were alloimmunized. The total number of alloantibodies identified in these patients was 25. Two patients (18%) had only one alloantibody, six patients (54.5%) had two alloantibodies, one patient (9%) had three alloantibodies, and two patients (18%) had more than three alloantibodies. The most prevalent antibodies belonged to Kell and Rh blood-group systems (seven out of 25 each). Conclusions When it comes to thalassemia patients dependent on blood transfusion, red blood cell alloimmunization is a difficult complication. Kell and RH blood-group-system antibodies are the predominant antibodies. Formation of alloantibodies depends mainly on the nature of red cell units received and donor and recipient factors. These can explain the difference in alloimmunization rates in different reports. Avoidance of alloimmunization would decrease the cost of treatment on the long run and improve the patient quality of life.\",\"PeriodicalId\":7866,\"journal\":{\"name\":\"Alexandria Journal of Pediatrics\",\"volume\":\"54 1\",\"pages\":\"26 - 32\"},\"PeriodicalIF\":0.0000,\"publicationDate\":\"2022-01-01\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Alexandria Journal of Pediatrics\",\"FirstCategoryId\":\"1085\",\"ListUrlMain\":\"https://doi.org/10.4103/ajop.ajop_5_22\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"\",\"JCRName\":\"\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Alexandria Journal of Pediatrics","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.4103/ajop.ajop_5_22","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"","JCRName":"","Score":null,"Total":0}
Red cell alloimmunization in transfusion-dependent β-thalassemia patients attending Alexandria University Children Hospital
Background In Egypt, it has been established that the most frequently inherited disorder of hemoglobin is β-thalassemia. Management of the severe forms of this disorder consists chiefly of red cell transfusion. The concerned patients receive these transfusions on a regular basis. Therefore, the formation of antired cell alloantibodies and autoantibodies was inevitable. This dilemma is addressed in this study that aims to determine the prevalence of red cell alloimmunization among transfusion-dependent ß-thalassemia children that receive frequent care in the Hematology Clinic of Alexandria University Children’s Hospital. Patients and methods This study screened 100 transfusion-dependent patients in total, all of them were 3–16 years old. Transfusion and clinical records of the latter were reviewed to assess the diagnosis of β-thalassemia, the age of the first presentation, the age at which transfusion therapy was started, transfusion interval, and the number of red cell units given. Alloantibody detection was then performed. Results In the present study, 11 (11%) out of the total 100 studied patients were alloimmunized. The total number of alloantibodies identified in these patients was 25. Two patients (18%) had only one alloantibody, six patients (54.5%) had two alloantibodies, one patient (9%) had three alloantibodies, and two patients (18%) had more than three alloantibodies. The most prevalent antibodies belonged to Kell and Rh blood-group systems (seven out of 25 each). Conclusions When it comes to thalassemia patients dependent on blood transfusion, red blood cell alloimmunization is a difficult complication. Kell and RH blood-group-system antibodies are the predominant antibodies. Formation of alloantibodies depends mainly on the nature of red cell units received and donor and recipient factors. These can explain the difference in alloimmunization rates in different reports. Avoidance of alloimmunization would decrease the cost of treatment on the long run and improve the patient quality of life.