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RESEARCH PRODUCT
HLA association is different in children and adults with severe acquired aplastic anemia
Cornelia DeppnerJürgen DurnerMonika FührerEkkehard D. AlbertHeiko GötteChristine Bender-götzeGünter Brünnlersubject
MaleAdolescentHuman leukocyte antigenmedicine.disease_causeAutoimmunityPathogenesisHLA-B14 AntigenHLA Antigenshemic and lymphatic diseasesmedicineHumansHLA-DR2 AntigenProspective StudiesChildAllelesbusiness.industryBone marrow failureAnemia AplasticInfantHematologymedicine.diseasePancytopeniaPathophysiologyExact testOncologyHLA-B AntigensChild PreschoolPediatrics Perinatology and Child HealthImmunologyCohortFemalebusinessdescription
Background Severe aplastic anemia (SAA) is defined as pancytopenia caused by bone marrow failure. The pathogenesis of SAA is thought to involve autoimmune processes. Increased susceptibility to autoimmunity has been shown to be associated with several different HLA alleles. In SAA, few large studies based on data mainly from adults describe a positive HLA correlation with HLA-DR2 (DRB1*15) and HLA-B14. Procedure This study explored the HLA constitution of 181 children with SAA who were enrolled in the prospective multi-center study SAA94 between January 1994 and January 2002. The control group consisted of 303 healthy individuals of comparable demographic background. Allelic frequencies between patients and controls are compared using Fisher's exact test. Results In our pediatric cohort, we describe a positive association with HLA-B14 (P = 0.0039), but no association of HLA-DR2 with SAA. Conclusion HLA associations appear to be different in children and adults with SAA. This might point towards a difference in pathophysiology between at least part of the children and adults. Pediatr Blood Cancer 2007;48:186–191. © 2006 Wiley-Liss, Inc.
year | journal | country | edition | language |
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2007-02-01 | Pediatric Blood & Cancer |