Response to Immunosuppressive Therapy and an HLA-DR Allele in Children with Aplastic Anemia / 대한혈액학회지

ABSTRACT

BACKGROUND: Genetic susceptibility to a variety of disease has been shown and it has recently been suggested that aplastic anemia is more common in adults who are HLA-DR2+ than in the general population. METHODS: To investigate whether certain HLA-DR gene is more common in children with aplastic anemia and HLA-DR2+ gene is associated with susceptibility to cyclosporine A (CyA), we analyzed the results of HLA-DR typing in 37 children with aplastic anemia with sequence specific oligonucleotide probe (SSOP) method. RESULTS: Eight patients were DR2+, and there was no more common than the normal population. All of the 8 patients with DR2+ were DRB1*1501+ with high resolution HLA typing. Of the 8 patients with DRB1*1501+, 3 patients were treated with CyA alone (2 patients) or CyA plus ATG (1 patient), 2 patients were treated with ATG (1 patient) or ALG alone (1 patient). Patients treated with CyA only or CyA plus ATG had either a complete (1 patient) or partial (2 patients) response and patients treated ATG or ALG alone had no response. CONCLUSION: Although incidence of HLA-DR2 was not significantly higher in children with aplastic anemia then normal population, response rate to CyA was significantly high in the DR2+ patients.