Dingli David, Schwager Susan M, Mesa Ruben A, Li Chin Yang, Tefferi Ayalew
Division of Hematology, Mayo Clinic College of Medicine, Rochester, Minnesota 55905, USA.
Cancer. 2006 Feb 1;106(3):623-30. doi: 10.1002/cncr.21644.
Allogeneic hematopoietic stem cell transplantation is potentially curative in agnogenic myeloid metaplasia (AMM) but is associated with substantial mortality and morbidity that necessitates accurate identification of patients in whom benefit outweighs risk. The current study describes the natural history of AMM in transplant-eligible patients and proposes a new prognostic scoring system that favorably compares with other established models.
Patients diagnosed with AMM before the age of 60 years and seen at Mayo Clinic were identified and the diagnosis confirmed. Relevant demographic, clinical, and laboratory characteristics were abstracted, and the impact of various parameters on overall survival (OS) was evaluated with univariate and multivariate analyses.
A cohort of 160 patients with AMM is described. OS was 78 months. Multivariate analysis identified a hemoglobin level of <10 g/dL, white blood cell count of either <4 or >30x10(9)/L, platelet count of <100x10(9)/L, presence of constitutional symptoms, and hepatomegaly as independent predictors of inferior survival. The first 3 complete blood count-based parameters were combined into a new scoring system that resulted in median survivals of 155, 69, and 24 months in the presence of 0, 1, or >or=2 adverse features. The chi-square value for the new model was 80.6 compared with 51.4, 48.4, and 43.7 for the models by Dupriez, Cervantes, and Visani, respectively.
A new scoring system based on blood count at the time of diagnosis can adequately stratify by risk transplant-eligible patients with AMM and can accurately identify high-risk as well as intermediate-risk disease. The new system displayed a stronger discriminative value, between risk categories, compared with currently existing prognostic models.
异基因造血干细胞移植对原发性骨髓纤维化（AMM）有潜在的治愈作用，但伴有较高的死亡率和发病率，因此有必要准确识别那些受益大于风险的患者。本研究描述了适合移植的AMM患者的自然病程，并提出了一种新的预后评分系统，该系统与其他已建立的模型相比具有优势。
确定在梅奥诊所就诊且年龄小于60岁、诊断为AMM的患者，并对诊断进行确认。提取相关的人口统计学、临床和实验室特征，通过单因素和多因素分析评估各种参数对总生存期（OS）的影响。
描述了一组160例AMM患者。OS为78个月。多因素分析确定血红蛋白水平<10 g/dL、白细胞计数<4或>30×10⁹/L、血小板计数<100×10⁹/L、存在全身症状和肝肿大是生存较差的独立预测因素。基于前3项全血细胞计数的参数被合并成一个新的评分系统，在存在0、1或≥2个不良特征时，中位生存期分别为155、69和24个月。新模型的卡方值为80.6，而Dupriez、Cervantes和Visani模型的卡方值分别为51.4、48.4和43.7。
基于诊断时血细胞计数的新评分系统可以充分地对适合移植的AMM患者进行风险分层，并能准确识别高危和中危疾病。与目前现有的预后模型相比，新系统在风险类别之间显示出更强的鉴别价值。
