title: Is the HLA-linked haemochromatosis allele implicated in idiopathic refractory sideroblastic anaemia?
abstract: In order to assess the previously reported association of HLA-linked idiopathic haemochromatosis with idiopathic refractory sideroblastic anaemia (IRSA), the prevalence of HLA-A3 antigen in a group of 22 patients with IRSA was compared to that observed in healthy controls and in patients with homozygous idiopathic haemochromatosis and to that calculated for a population heterozygous for idiopathic haemochromatosis. The prevalence of A3 in patients with IRSA (0.23) was quite similar to that observed in controls (0.29) and significantly different from that observed in homozygous (0.73; P less than 10(-5] and heterozygous (0.57; P less than 10(-3] haemochromatosis. Serum iron, transferrin saturation, serum ferritin and liver iron concentration showed no difference in IRSA patients with or without A3. It is concluded that there is neither systematic association between the haemochromatosis allele and IRSA nor systematic implication of such an allele in the development of iron overload observed in IRSA.
mesh_terms: D000368:Aged; D000483:Alleles; D000756:Anemia, Sideroblastic / Q000235:genetics / Q000276:immunology* / Q000378:metabolism; D005260:Female; D006680:HLA Antigens / Q000032:analysis* / Q000235:genetics; D015234:HLA-A Antigens; D015791:HLA-A3 Antigen; D015235:HLA-B Antigens; D006432:Hemochromatosis / Q000235:genetics / Q000276:immunology* / Q000378:metabolism; D006801:Humans; D007501:Iron / Q000378:metabolism; D008297:Male; D008875:Middle Aged
chemical_list: D006680:HLA Antigens; D015234:HLA-A Antigens; D015791:HLA-A3 Antigen; D015235:HLA-B Antigens; D007501:Iron