HLA-DR5 and DQB1*03 class II alleles are associated with cutaneous T-cell lymphoma.

Cutaneous T-cell lymphoma (CTCL) may present with eczematous lesions, mycosis fungoides (MF), or as exfoliative erythroderma with circulating atypical cells, Sezary syndrome (SS). The "malignant" T cells are epidermotropic and clonal, but whether they respond to antigen stimulation is unknown. Because CD4+ lymphocytes recognize antigen presented by histocompatibility locus antigen (HLA) class II molecules, and HLA associations have been found in autoimmune skin diseases, we determined by allele-specific oligonucleotide typing whether HLA-DR or DQ alleles were associated with CTCL and its two variants MF (n = 47) and SS (n = 23). Phenotypic frequencies were compared by chi-square and Fisher exact test, and p values were corrected independently for either 12 DR or 15 DQ alleles. HLA-DR5, previously associated with MF, was significantly increased in all 70 CTCL patients (31.5%) versus controls (11%) (uncorrected p value [p nc ] = 0.000038, odds ratio [OR] = 3.9, 1.9 nc ] = 0.000047, OR=3.62, 1.9 nc ] = 0.03, OR=3, 0.9 versus 49% of controls (corrected p value [p c ] = 0.014, OR=2.7, 1.4 c ] = 0.05, OR=4.7, 1.4 nc ] = 0.024, OR=2.15, 1 c ] = 0.045, OR 7.75, 1.25