Leczenie wenetoklaksem młodego pacjenta z przewlekłą białaczką limfocytową z delecją 17p i nawrotem choroby po pierwszej linii leczenia — opis przypadku i przegląd literatury

Q4 Medicine Hematologia Pub Date : 2020-01-01 DOI:10.5603/HEM.2020.0044
A. Dąbrowska-Iwanicka, Katarzyna Błachnio, Renata Woroniecka, Grzegorz Rymkiewicz, J. Walewski
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Abstract

Chronic lymphocytic leukaemia (CLL) is the commonest B-cell malignancy and leukaemia in western countries. It mainly affects the elderly population with a median age of 72. Overall survival varies from 2 to 15 years and depends on clinical and pathological risk factors like age, clinical stage and the presence of genetic mutations with TP53 aberrations conferring the worst prognosis. Indications for therapy include active and symptomatic disease and advanced clinical stage assessed in the Rai or Binet staging system. CLL therapy has been revolutionized in the last years due to the introduction of novel agents — small molecule inhibitors interfering with signalling pathways. These agents proved to be of greater efficacy than immunochemotherapy in both untreated and relapsed patients and led to the significant improvement in survival of CLL patients. These novel agents have been incorporated into new recommendations for CLL treatment. They have also improved the survival of high-risk CLL patients with TP53 aberrations and complex karyotype although their prognosis is still inferior as compared to other patients’ subgroups. Questions concerning optimal sequencing novel agents and the timing of allogeneic bone marrow transplantation remain still open for high-risk group CLL patients.
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慢性淋巴细胞白血病(CLL)是西方国家最常见的b细胞恶性肿瘤和白血病。主要影响年龄中位数为72岁的老年人。总生存期从2年到15年不等,取决于临床和病理风险因素,如年龄、临床分期和TP53异常基因突变的存在,这些因素会导致最坏的预后。治疗的适应症包括活动性和症状性疾病,以及在Rai或Binet分期系统中评估的晚期临床阶段。CLL治疗在过去几年中已经发生了革命性的变化,因为引入了新的药物-干扰信号通路的小分子抑制剂。事实证明,这些药物在未治疗和复发患者中都比免疫化疗更有效,并显著提高了CLL患者的生存率。这些新型药物已被纳入CLL治疗的新建议。他们也提高了TP53畸变和复杂核型的高危CLL患者的生存率,尽管与其他患者亚组相比,他们的预后仍然较差。高风险CLL患者的最佳药物排序和同种异体骨髓移植的时机问题仍然存在。
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来源期刊
Hematologia
Hematologia Medicine-Oncology
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审稿时长
4 weeks
期刊介绍: Hematology is the quarterly under auspices of the Institute of Hematology and Transfusion Medicine. The journal is addressed to hematologists, oncologists and also internists. It contains the overview/review articles, case reports, essays, including reports from the scientific and educational conferences as well as test questions on hematology. Journal of the Institute of Hematology and Transfusiology.
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