Objective To investigate the clinical features of AIHA/ES patients with clonal Ig/TCR gene rearrangement. Methods Ig/TCR gene rearrangements were measured by BIOMED-2 PCR. 44 primary AIHA/ES patients were enrolled in the study. Clinical characteristics were analyzed and compared between patients with and without clonal Ig/TCR gene rearrangement. Results Clonal Ig/TCR rearrangements were identified in 34.09% (15/44) patients including 18.18% (8/44) clonal Ig rearrangement and 15.91% (7/44) clonal TCR rearrangement. 11.37% (5/44) patients showed TCR gamma rearrangement, and 2.27% (1/44) patient showed beta rearrangement. 2.27% (1/44) patient showed both gamma and beta chain rearrangement. The median ages of patients with Ig/TCR clonality (8 male and 7 female) and without Ig/TCR clonality (10 male and 19 female) were 60 (16 similar to 81) and 53 (17 similar to 78) years old, respectively. No significant differences were found in age or gender between the two groups (p = .26,p = .378). Hb and RBC of patients with Ig/TCR clonality [(64.31 +/- 5.72) g/L, (1.78 +/- 0.22) x 10(12)/L] were significantly lower than those of patients without Ig/TCR clonality (p = .0053 andp = .0189, respectively). The percentage of reticulocytes of Ig/TCR clonality group was obviously higher than that of patients without Ig/TCR clonality (p = .0248). No significant differences were found in levels of TBIL, IBIL, LDH, FHb, Hp, IgG, IgA, IgM, IgE, C3, C4, CD5(+)CD19(+)/CD19(+)ratio, and CD3(+)CD4(+)/CD3(+)CD8(+)ratio between the two groups. Treatment response of Ig/TCR clonality group occurred significantly later than that of the non-clonality group (p = .0016). There were no differences in relapse rate, time to recurrence, and duration of remission between two groups (p = .083,p = .72, andp = .61, respectively). Conclusion AIHA/ES patients with clonal Ig/TCR gene rearrangement presented more severe haemolysis and anaemia. Longer treatment is needed for these patients to obtain remission.