Sickle cell leg ulcers are associated with HLA-B35 and Cw4.
We determined the HLA-A, B, C, and DR types in nine patients with sickle cell anemia (SS) who had leg ulcers or a history of leg ulcers, and in 29 control patients with SS without leg ulcers. Six (67%) of the nine patients with leg ulcers had HLA-B35 and each of these six patients also had HLA-Cw4. In contrast, only eight (28%) of the 29 control patients with SS had HLA-B35 and only three (10%) of these patients had both HLA-B35 and Cw4. The relative risk for development of leg ulcers in patients with SS who had both HLA-B35 and Cw4 was 17 times greater than that of patients without these antigens or who had only one antigen. The frequency of HLA-B35 was also significantly higher in patients with SS and leg ulcers than in a reference population (31%) consisting of 68 healthy black persons. These results suggest that genetic factors or an HLA-related altered immune response may contribute to the development of leg ulcers in sickle cell anemia.
D000293:Adolescent; D000328:Adult; D000755:Anemia, Sickle Cell / Q000276:immunology*; D005260:Female; D006680:HLA Antigens / Q000032:analysis*; D015797:HLA-B35 Antigen; D015236:HLA-C Antigens; D006801:Humans; D007871:Leg Ulcer / Q000276:immunology*; D008297:Male; D008875:Middle Aged
D006680:HLA Antigens; D015797:HLA-B35 Antigen; D015236:HLA-C Antigens; C039966:HLA-C*04 antigen