Abstract

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 γ rearrangement, and 2.27% (1/44) patient showed β rearrangement. 2.27% (1/44) patient showed both γ and β 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 ∼ 81) and 53 (17 ∼ 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) × 10¹²/L] were significantly lower than those of patients without Ig/TCR clonality (p = .0053 and p = .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, and p = .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.

中文翻译：

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克隆性免疫球蛋白/T细胞受体基因重排自身免疫性溶血性贫血/Evans综合征患者的临床特征

摘要

客观的

探讨克隆性Ig/TCR基因重排AIHA/ES患者的临床特征。

方法

Ig/TCR 基因重排通过 BIOMED-2 PCR 测量。44 名原发性 AIHA/ES 患者参加了该研究。分析和比较有无克隆性 Ig/TCR 基因重排患者的临床特征。

结果

在 34.09% (15/44) 的患者中鉴定出克隆 Ig/TCR 重排，包括 18.18% (8/44) 克隆 Ig 重排和 15.91% (7/44) 克隆 TCR 重排。11.37% (5/44) 患者显示 TCR γ 重排，2.27% (1/44) 患者显示 β 重排。2.27% (1/44) 的患者同时出现 γ 和 β 链重排。Ig/TCR克隆性患者（8男7女）和无Ig/TCR克隆性患者（10男19女）的中位年龄分别为60岁（16～81岁）和53岁（17～78岁）。两组之间的年龄或性别没有显着差异（p  = .26，p  = .378）。Ig/TCR 克隆性患者的 Hb 和 RBC [(64.31 ± 5.72) g/L, (1.78 ± 0.22) × 10 ¹²/L] 显着低于无 Ig/TCR 克隆性的患者（分别为p  = .0053 和p  = .0189）。Ig/TCR 克隆 组网织红细胞百分比明显高于无 Ig/TCR 克隆组（p = .0248）。TBIL、IBIL、LDH、FHb、Hp、IgG、IgA、IgM、IgE、C3、C4、CD5 ⁺ CD19 ⁺ /CD19 ⁺比值、CD3 ⁺ CD4 ⁺ /CD3 ⁺ CD8 ⁺比值水平无显着差异两组之间。Ig/TCR 克隆组的治疗反应发生明显晚于非克隆组（p = .0016）。两组在复发率、复发时间和缓解持续时间方面没有差异（分别为p  = .083、p  = .72 和p  = .61）。

结论

具有克隆性 Ig/TCR 基因重排的 AIHA/ES 患者出现更严重的溶血和贫血。这些患者需要更长时间的治疗才能获得缓解。