伊斯帕塔慢性髓性白血病患者的真实生活数据

Murat Ardoğan, Demircan Özbalci, Emine Güçhan Alanoğlu
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摘要

目的:慢性髓性白血病是一种骨髓增生性肿瘤,发病率为每 10 万名成人中 1-2 例。CML发病机制的核心是9号染色体上的阿贝尔森鼠白血病(ABL1)基因与22号染色体上的断点集群区(BCR)基因的融合。随着酪氨酸激酶抑制剂(TKIs)的开发,治疗格局发生了巨大变化。这种 "靶向 "方法改变了 CML 的自然病史,将 10 年生存率提高到 80%-90% 。本研究旨在调查 TKI 治疗的有效管理和 "现实生活中 "的 CML 患者的总生存率,并将结果与现有文献进行讨论。 材料与方法:研究人员评估了苏莱曼-德米雷尔大学(Süleyman Demirel University)血液科 2000 年至 2018 年期间确诊为 CML 的 88 例患者。本研究评估了患者的临床和实验室特征、临床和人口统计学特征、治疗方案、副作用和反应。风险评估和分期采用了世界卫生组织的标准以及索卡尔-哈斯福德(Sokal Hasford)和尤托斯(Eutos)风险评分系统。我们根据欧洲白血病网络标准确定了血液学、细胞遗传学和分子反应。 结果:平均年龄、男女比例和存活率与文献报道相似。年龄分布(p = 0.001)、Charlson合并症指数(p = 0.005)和Charlson合并症-年龄指数(p = 0.000)对总生存率有显著的统计学影响。年龄分布(p = 0,029)、Charlson 合并症年龄指数(p = 0,001)和 12 个月时的主要分子反应(p = 0,028)对无病生存期有显著影响。12 个月时的主要分子反应(p = 0,006)对无进展生存期也有显著的统计学影响。网状纤维分级对患者的总生存期、无病生存期和无进展生存期没有明显影响。 结论:这些结果表明,通过现有的治疗方案,CML 一般都能得到很好的控制,而死亡更多是由于其他医疗问题。在 CML 中,Charlson 指数已被证明与总生存率和无病生存率显著相关。
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Isparta’daki Kronik Myeloid Lösemi Hastalarının Gerçek Yaşam Verileri
OBJECTIVE: Chronic myeloid leukemia is a myeloproliferative neoplasm with an incidence of 1–2 cases per 100 000 adults. Central to the pathogenesis of CML, is the fusion of the Abelson murine leukemia (ABL1) gene on chromosome 9 with the breakpoint cluster region (BCR) gene on chromosome 22. The therapeutic landscape changed dramatically with the development of the tyrosine kinase inhibitors (TKIs). This “targeted” approach altered the natural history of CML, improving the 10-year survival rate to 80 %-90 %. This study aims to investigate the effective management of TKI treatment and overall survival in “real-life” CML patients and to discuss the results with current literature. MATERIAL AND METHODS: Eighty eight patients who were diagnosed as CML between 2000 and 2018 in Süleyman Demirel University Hematology Department were evaluated. Patients’ clinical and laboratory characteristics, clinical and demographical features, treatment options, side effects and responses were evaluated in this study. Risk assessment and staging applied with World Health Organization criteria and Sokal Hasford and Eutos risk scoring system. We determined hematological, cytogenetic and molecular response according to European Leukemia Network criteria. RESULTS: The average age, male / female ratio and survival rates were found similar to those in literature. The age distribution (p = 0,001) and Charlson comorbidity index (p = 0.005) and Charlson comorbidity-age index (p = 0,000) had a statistically significant effect on overall survival. Age distribution (p = 0,029), Charlson comorbidity age index (p = 0,001) and major molecular response at 12 months (p = 0,028) were found to have a significant effect on disease-free survival. Major molecular response at 12 months (p = 0,006) also had a statistically significant effect on progression-free survival. Reticular fiber grade did not significantly affect overall survival, disease-free survival and progression-free survival of patients. CONCLUSION: These results suggest that CML is generally well managed with existing treatment options and that death occur more frequently due to other medical problems. In CML, Charlson indices have been shown to be significantly associated with overall survival and disease-free survival.
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