Platelet-derived growth factor receptor alpha (PDGFRA) is important in numerous malignancies and can serve as a target for therapeutic strategy. We aimed to assess the role of PDGFRA expression in mature T- and natural killer (NK)-cell lymphomas. We used immunohistochemistry to analyze PDGFRA expression in mature T- and NK-cell lymphomas in tissue samples from 50 patients. In positive samples, we then did a mutational analysis of the PDGFRA gene on exons 10, 12, 14, and 18. The relationship between PDGFRA expression and overall survival in mature T- and NK-cell lymphomas was statistically analyzed in the study. We analyzed PDGFRA expression in four subtypes: angioimmunoblastic T-cell lymphoma (3/8, 37.5%); anaplastic large cell lymphoma (5/7, 71.4%); NK/T-cell lymphoma, nasal type (9/12, 75%); and peripheral T-cell lymphoma, unspecified (PTCLu; 7/23, 30.4%). It was lower in PTCLu than in other subtypes (30.4% vs. 63%, p=0.022). PDGFRA expression was not related to PDGFRA gene mutation. Overall survival in mature T- and NK-cell lymphomas correlated significantly with disease subtypes and an international prognostic index but not PDGFRA expression. Our study showed that PDGFRA expression was different in mature T- and NK-cell lymphomas. PDGFRA expression in PTCLu was lower in the present study than in previous reports done in Western countries. It suggests that this disease is biologically distinct in different races (30.4% vs. 91-100%).