Hematology, ASST Spedali Civili di Brescia, Brescia, Italy.
Clinical Chemistry Laboratory, Diagnostic Department, ASST Spedali Civili Di Brescia, Brescia, Italy.
Ann Hematol. 2022 Jun;101(6):1227-1237. doi: 10.1007/s00277-022-04806-x. Epub 2022 Apr 5.
Therapy-related myeloid neoplasm (t-MN) is a threatening complication of autologous stem cell transplantation (ASCT). Detecting clonal hematopoiesis (CH) mutations in cryopreserved cells before ASCT has been associated with a higher risk of t-MN, but the evolution of molecular abnormalities from pre-ASCT to t-MN, within the same patient, remains to be elucidated. We evaluated the mutational profile of 19 lymphoma/myeloma patients, at both pre-ASCT and t-MN diagnosis, using a targeted NGS approach; 26 non-developing t-MN control patients were also studied pre-ASCT. At ASCT, we found a higher frequency of CH in patients developing t-MN (58%) than in those who did not (23%) (P = 0.029); mutations in epigenetic (DNMT3A, TET2, and ASXL1) and DNA repair genes (PPM1D, RAD21, TP53, and STAG2) were the most represented. At t-MN, CH increased to 82% of patients. Cumulative mutational burden and variant allele frequency (VAF) also increased at t-MN. CH clones detected at ASCT were found at t-MN in eight out of 16 patients, mainly with stable VAF. Among the new driver mutations appeared at t-MN, TP53 increased from one to 13 mutations, in nine patients; being associated with complex karyotype. Mutations in transcription factor (RUNX1, CEBPA) and intracellular signaling genes (FLT3, RAS genes) also increased from three to 17 mutations in eight patients, presenting with a normal karyotype. Overall, we found that preexisting CH at ASCT rarely causes t-MN directly, but may rather facilitate the appearance of new mutations, especially those involving TP53, RUNX1, and RAS, that can drive the evolution to t-MN of at least two distinct types.
治疗相关的髓系肿瘤(t-MN)是自体干细胞移植(ASCT)的一种威胁生命的并发症。在 ASCT 前检测冷冻细胞中的克隆性造血(CH)突变与 t-MN 风险增加相关,但同一患者从 ASCT 前到 t-MN 的分子异常演变仍有待阐明。我们使用靶向 NGS 方法评估了 19 例淋巴瘤/骨髓瘤患者在 ASCT 前和 t-MN 诊断时的突变谱;还研究了 26 例非进展性 t-MN 对照患者的 ASCT 前情况。在 ASCT 时,我们发现发生 t-MN 的患者中 CH 的频率(58%)高于未发生的患者(23%)(P=0.029);表观遗传(DNMT3A、TET2 和 ASXL1)和 DNA 修复基因(PPM1D、RAD21、TP53 和 STAG2)的突变最为常见。在 t-MN 时,CH 增加到 82%的患者。在 t-MN 时,累积突变负担和变异等位基因频率(VAF)也增加。在 16 例患者中的 8 例中,在 ASCT 时检测到的 CH 克隆在 t-MN 中被发现,主要是 VAF 稳定。在 t-MN 中出现的新驱动突变中,TP53 从 1 个增加到 9 个患者的 13 个突变,与复杂核型相关。转录因子(RUNX1、CEBPA)和细胞内信号基因(FLT3、RAS 基因)的突变也从 3 个增加到 8 个患者的 17 个突变,表现为正常核型。总体而言,我们发现 ASCT 时存在的预先存在的 CH 很少直接导致 t-MN,但可能更容易出现新的突变,特别是那些涉及 TP53、RUNX1 和 RAS 的突变,这些突变可以至少导致两种不同类型的 t-MN 进化。