Korean J Hematol.  1999 Feb;34(1):36-42.

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

Affiliations
  • 1Department of Pediatrics, College of Medicine, Yonsei University, Seoul, Korea.
  • 2Department of Microbiology, College of Medicine, Yonsei University, Seoul, Korea.
  • 3Department of Pediatrics, College of Medicine, Hallym University, Korea.
  • 4Department of Pediatrics, College of Medicine, Ajou University, Korea.
  • 5Department of Pediatrics, College of Medicine, Yonsei University Wonju College of Medicine, Korea.

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.

Keyword

Aplastic anemia; HLA-DR; Immunosuppresive therapy

MeSH Terms

Adult
Alleles*
Anemia, Aplastic*
Child*
Cyclosporine
Genetic Predisposition to Disease
Histocompatibility Testing
HLA-DR Antigens*
HLA-DR2 Antigen
Humans
Incidence
Cyclosporine
HLA-DR Antigens
HLA-DR2 Antigen
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