The outcomes of patients with acute myeloid leukemia (AML) and bone marrow fibrosis (MF) are not well defined. The study objectives were to evaluate the degrees of MF in AML, and corresponding response rates and outcomes. We performed a retrospective review of 2302 patients with AML. We annotated the clinical and molecular characteristics, response to therapy, and survival outcomes of patients with bone marrow fibrosis. Overall, 492 patients (21.4%) had a reported microscopic evaluation of MF: 344 (69.9%) had MF grade 0–1 and 148 (30.1%) had MF grade 2–3. Patients with MF 2–3 had a higher proportion of complex cytogenetics (39.2% vs. 24.7%, p = 0.002) JAK2 mutations (25.7% vs. 18%, p = 0.07) and lower proportion of IDH2 (16.9% vs. 25.9%, p = 0.03) and CEBPA (15.5% vs. 27.6%, p = 0.006) mutations. 64% were treated with low-intensity chemotherapy (LIT) and 36.1% with intensive chemotherapy (IT). The complete remission (CR)/CR with incomplete count recovery (CRi) rates were 63.5% with IC versus 37.9% with LIT (p = 0.007). In patients aged 60 or older 4-week mortality was 12.5% with IC vs. 9.3% with LIT (p = 0.8). The median overall survival (OS) was 14.2 with MF 0–1 versus 7.5 months with MF 2–3 (p < 0.005). In patients aged 60 or older with MF 2–3 median OS was 6.5 months with IT versus 7.0 months with LIT (p = 0.19). In a multivariate analysis, grade 2–3 MF (HR 2.0, 95%CI 1.59–2.51) was the strongest prognostic factor for survival. In summary, grade 2–3 MF in AML is associated with worse outcomes.

中文翻译：

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急性髓系白血病合并骨髓纤维化患者的结局


急性髓系白血病 （AML） 和骨髓纤维化 （MF） 患者的结局尚不明确。研究目的是评估 AML 中 MF 的程度，以及相应的反应率和结果。我们对 2302 例 AML 患者进行了回顾性评价。我们注释了骨髓纤维化患者的临床和分子特征、对治疗的反应和生存结局。总体而言，492 例患者 （21.4%） 报告了 MF 的显微镜评估：344 例 （69.9%） 的 MF 分级为 0-1 级，148 例 （30.1%） 的 MF 分级为 2-3 级。MF 2-3 患者具有较高比例的复杂细胞遗传学 （39.2% vs. 24.7%，p = 0.002） JAK2 突变 （25.7% vs. 18%，p = 0.07） 和 IDH2 （16.9% vs. 25.9%，p = 0.03） 和 CEBPA （15.5% vs. 27.6%，p = 0.006） 突变的比例较低。64% 接受低强度化疗 （LIT） 治疗，36.1% 接受强化化疗 （IT）。IC 组的完全缓解 （CR）/CR 不完全计数恢复 （CRi） 率为 63.5%，LIT 组为 37.9% （p = 0.007）。在 60 岁或以上的患者中，IC 组的 4 周死亡率为 12.5%，LIT 组为 9.3% （p = 0.8）。MF 0-1 的中位总生存期 （OS） 为 14.2，而 MF 2-3 为 7.5 个月 （p < 0.005）。在 60 岁或以上的 MF 2-3 患者中，IT 组的中位 OS 为 6.5 个月，LIT 组为 7.0 个月 （p = 0.19）。在多变量分析中，2-3 级 MF （HR 2.0,95%CI 1.59-2.51） 是最强的生存预后因素。总之，AML 中的 2-3 级 MF 与较差的结果相关。