However, the diverse etiologies of acquired PRCA make its pathogenesis largely unclear.<h4>Materials and methods</h4>We portrayed the clinicopathologic, mutation and TCR rearrangement profiles of 64 primary PRCA cases and 104 large granular lymphocytic leukemia (LGLL)-associated PRCA, and tried to reveal the association factors of CsA response.<h4>Results</h4>We found that gene mutations were detected in 39.7% of acquired PRCA who were older than 40 years, with DNMT3A, KMT2A and TP53 being the top 3 mutation genes. The gene discussed is KMT2A; the disease is T-cell large granular lymphocyte leukemia.