Paterno Giovangiacinto, Palmieri Raffaele, Tesei Cristiano, Nunzi Andrea, Ranucci Giorgia, Mallegni Flavia, Moretti Federico, Meddi Elisa, Tiravanti Ilaria, Marinoni Massimiliano, Page Camilla, Fagiolo Solaria, Buzzatti Elisa, Secchi Roberto, Gurnari Carmelo, Maurillo Luca, Buccisano Francesco, Venditti Adriano, Del Principe Maria Ilaria
Hematology, Fondazione Policlinico Tor Vergata, Rome, Italy.
Hematology, Department of Biomedicine and Prevention, University of Tor Vergata, Rome, Italy.
Thromb Res. 2024 Apr;236:30-36. doi: 10.1016/j.thromres.2024.02.017. Epub 2024 Feb 16.
Coagulation disorders frequently complicate the clinical course of acute myeloid leukemia (AML) patients. This study examined the frequency and prognostic significance, with regards of early mortality, of the presence of overt disseminated intravascular coagulation (DIC) at AML diagnosis and its correlation with clinical and biological characteristics. A retrospective analysis of 351 newly diagnosed non-promyelocytic AML patients was conducted, utilizing the 2018 ISTH DIC-Score criteria to evaluate the presence of overt DIC at AML onset. The study cohort had a median age of 65 years with a predominance of male gender (59 %). Overt DIC was present in 21 % of cases and was associated with advanced age, comorbidities, poor performance status, hyperleukocytosis, LDH levels, NPM1 mutations, expression of CD33 and CD4, and lack of expression of CD34. With a median follow-up of 72 months (3-147 months), the 6-year overall survival (OS) was 17.4 %, with patients having overt DIC showing significantly poorer outcomes (7.2 % compared to 20.3 % of those without DIC, p < 0.001). Patients with overt DIC showed markedly high early mortality rates at 30 (42.5 % vs 8 %), 60 (49.3 % vs 16.9 %), and 120 days (64.4 % vs 25.6 %) from disease onset. In multivariate analysis overt DIC retained its independent prognostic value for early mortality. In conclusion, the prevalence and clinical relevance of DIC in non-promyelocytic AML is not negligible, underlining its potential as an unfavorable prognostic marker. In newly diagnosed patients with AML, early recognition and measure to counteract coagulation disturbances might help mitigate the elevated mortality risk associated with DIC.
凝血功能障碍常使急性髓系白血病（AML）患者的临床病程复杂化。本研究探讨了AML诊断时明显的弥散性血管内凝血（DIC）的发生率及其对早期死亡率的预后意义，以及它与临床和生物学特征的相关性。对351例新诊断的非早幼粒细胞性AML患者进行了回顾性分析，采用2018年国际血栓与止血学会（ISTH）的DIC评分标准评估AML发病时明显DIC的存在情况。研究队列的中位年龄为65岁，男性占多数（59%）。21%的病例存在明显DIC，且与高龄、合并症、体能状态差、白细胞增多、乳酸脱氢酶（LDH）水平、核仁磷酸蛋白1（NPM1）突变、CD33和CD4的表达以及CD34表达缺失有关。中位随访72个月（3 - 147个月），6年总生存率（OS）为17.4%，有明显DIC的患者预后明显较差（7.2%，而无DIC患者为20.3%，p < 0.001）。有明显DIC的患者在疾病发作后30天（42.5%对8%）、60天（49.3%对16.9%）和120天（64.4%对25.6%）的早期死亡率明显较高。在多变量分析中，明显DIC对早期死亡率仍具有独立的预后价值。总之，DIC在非早幼粒细胞性AML中的患病率和临床相关性不可忽视，突显了其作为不良预后标志物的潜力。在新诊断的AML患者中，早期识别和应对凝血紊乱的措施可能有助于降低与DIC相关的升高的死亡风险。
