الفهرس 
IntroductionOnly 14 pages are availabe for public view
 |  | from | 110 |  |  |
| | | from | 110 | | |
Abstract
Background and objective: AIHA is characterized by presence of autoantibodies that bind to the erythrocyte surface membrane and lead to premature red cell destruction.CTLA4 has an important role in a negative regulation of cell responses by reducing CD 28/B7 interaction because its higher affinity for B7. Polymorphism at A to G polymorphism at position 49 of theCTLA4 first exon. Aim of our work was assessment of clinical spectrum, management and outcome of children with IAIHA following up with hematology outpatient clinic, Cairo University and Benisuef Pediatric hospital as well as newly diagnosed ones over the next 6 months and to investigate the frequency of CTLA4 G allele and its possible contribution in the pathogenesis of AIHA in children.
Subjects and methods: A retroprospective study was recruiting 20 patients with primary AIHA by taking complete history ,laboratory investigation including C.B.C, indirect serum bilirubin, reticulocyte assay, direct antiglobulin test ,urine analysis, hepatitis C virus and PCR to detect frequency of CTLA449G allele in patients as well as in 20 healthy control children .The progress of the disease and the different lines of treatment needed were also assessed.
Results:This study reported difficulty of cross matching in whole our patients when blood transfusion was done. Median hemoglobin level was 5.45g/dl and the median reticulocyte % was 13.95%, positive direct Coombs test in 75%. All of our patients received oral steroids as 1st line of treatment followed by I.V steroids in patients with unacceptable Hb levels under oral steroids. Immunosuppressive drug (e.g azathioprine) was used in 25% of patients whose poor response to steroids. Splenectomy was performed in 2 of our cases whose hemolytic process remained uncontrollable despite the above measures. Acute course of IAIHA was more in teenage than infant group who prone to a chronic course of illness,CTLA4A49G allele was in higher level in patients than control group.
Conclusion: CTLA4 A49G polymorphism is associated with occurrence of IAIHA. G allele is reported in both patients and controls but more predominant among patients. Immunosuppressive drugs are effective in treatment of resistant cases, few patients unresponsive to all therapeutic maneuvers.