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P53基因重排/突变的白血病非淋巴结套细胞淋巴瘤对利妥昔单抗/伊布替尼组合疗法高度敏感

2018-03-31 MedSci MedSci原创

白血病型非淋巴结套细胞淋巴瘤(L-NN-MCL)是一种相对罕见的套细胞淋巴瘤亚型,通常被认为是无痛性的。尽管对L-NN-MCL的深入认识能够避免过度治疗,但部分患者具有更激进的L-NN-MCL形式并呈现出症状性疾病和/或血细胞减少症。在此,研究人员为我们展示了两例需要进一步治疗的侵袭性L-NN-MCL案例。两例患者的细胞遗传学和DNA测序研究显示17号染色体或P53基因异常,两例患者均接受利妥昔单

白血病型非淋巴结套细胞淋巴瘤L-NN-MCL)是一种相对罕见的套细胞淋巴瘤亚型,通常被认为是无痛性的。尽管对L-NN-MCL的深入认识能够避免过度治疗,但部分患者具有更激进的L-NN-MCL形式并呈现出症状性疾病和/或血细胞减少症。在此,研究人员为我们展示了两例需要进一步治疗的侵袭性L-NN-MCL案例。两例患者的细胞遗传学和DNA测序研究显示17号染色体或P53基因异常,两例患者均接受利妥昔单抗联合伊布替尼治疗,联合治疗方案能够在2个月内使患者的WBCALC,血红蛋白和血小板正常化,并在自体干细胞移植(ASCT)后获得完全缓解(CR)。

这两例患者分别为:5年无症状淋巴细胞增多症史的53L-NN-MCL男性患者,其白细胞迅速恶化,并发展为贫血和血小板减少症,17号染色体异常提示P53基因缺失;49L-NN-MCL男性患者,其WBC升高,绝对淋巴细胞增多,基因组测序显示TP53KMT2AMLL)基因中的错义突变以及BCOR中的移码突变。这两例患者对利妥昔单抗/伊布替尼联合治疗高度敏感。

原始出处:

Mori, Shahram, et al. "Aggressive Leukemic Non-Nodal Mantle Cell Lymphoma with P53 Gene Rearrangement/Mutation is Highly Responsive to Rituximab/Ibrutinib Combination Therapy." Biology of Blood and Marrow Transplantation 2018 24.3: S264-S265. doi.org/10.1016/j.bbmt.

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    2019-12-24 肖诗瑶

    bcor突变对套细胞淋巴瘤的意义及影响有哪些

    0

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    2018-04-02 sodoo
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    2018-04-02 zhishijing
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    2018-04-02 lqvr

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