我们在 essential thrombocythemia（ET）中研究了绝对中性粒细胞计数（ANC）、淋巴细胞计数（ALC）和单核细胞计数（AMC）对总体生存（OS）、无白血病生存（LFS）和无骨髓纤维化生存（MFFS）的个体预后贡献。我们从梅奥诊所数据库中回顾性收集了具有临床意义的病例（N=598；中位年龄59岁；女性占62%）：JAK2占59％，CALR占27％，三阴性占11％，MPL占3％；ET国际预后评分系统（IPSET）分类为高风险21％，中风险42％和低风险37％；7％（37/515）的患者具有异常核型，10％（21/205）的患者具有不良突变（SF3B1/SRSF2/U2AF1/TP53）。在中位8.4年的随访期内，记录了163例（27％）死亡，71例（12％）纤维化转化和20例（3％）白血病转化。多变量分析的结果显示：年龄＞70岁的HR（95％CI）为16.5（9.9-27.4），年龄50-70岁的HR为3.7（2.3-6.0），ANC≥8×109 /L的HR为2.4（1.7-3.3），ALC＜1.7×109 /L的HR为1.9（1.4-2.6）。对应的基于HR的评分分别为4、2、1和1，形成了一个新的四分级AgeAncAlc（AAA；三个A）风险模型：高风险（5-6分，中位生存8年，HR 30.1，95％CI 17.6-54）、中等风险2（4分，中位生存13.5年，HR 12.7，95％CI 7.1-23.0）、中等风险1（2-3分，中位生存20.7年，HR 3.8，95％CI 2.3-6.4）和低风险（0-1分，中位生存47年）。该AAA模型（Akaike信息准则[AIC] 621）的表现优于IPSET（AIC 647），并通过外部佛罗伦萨大学ET队列（N=485）进行了验证。AAA变量中没有一个预测了LFS，而ALC＜1.7×109 /L与较差的MFFS相关（p=.01）。不良突变（p＜.01）和核型（p＜.01）显示出额外的预后价值，但并未破坏AAA模型的预测能力。本研究提出了一个简单且全球适用的ET生存模型，可用作进一步分子精细化的基础。本研究还提出了免疫相关生物标志物作为骨髓增生性肿瘤预后工具的潜在作用。©2023 The Authors. 由Wiley Periodicals LLC出版的《美国血液学杂志》 发表。

We examined the individual prognostic contribution of absolute neutrophil (ANC), lymphocyte (ALC), and monocyte (AMC) counts, on overall (OS), leukemia-free (LFS), and myelofibrosis-free (MFFS) survival in essential thrombocythemia (ET). Informative cases (N = 598; median age 59 years; females 62%) were retrospectively accrued from a Mayo Clinic database: JAK2 59%, CALR 27%, triple-negative 11%, and MPL 3%; international prognostic scoring system for ET (IPSET) risk high 21%, intermediate 42%, and low 37%; 7% (37/515) had abnormal karyotype and 10% (21/205) adverse mutations (SF3B1/SRSF2/U2AF1/TP53). At median 8.4 years, 163 (27%) deaths, 71 (12%) fibrotic, and 20 (3%) leukemic transformations were recorded. Multivariable analysis resulted in HR (95% CI) of 16.5 (9.9-27.4) for age > 70 years, 3.7 (2.3-6.0) for age 50-70 years, 2.4 (1.7-3.3) for ANC ≥8 × 109 /L, and 1.9 (1.4-2.6) for ALC <1.7 × 109 /L. The corresponding HR-based scores were 4, 2, 1, and 1, resulting in an new 4-tiered AgeAncAlc (AAA; triple A) risk model: high (5-6 points; median survival 8 years; HR 30.1, 95% CI 17.6-54), intermediate-2 (4 points; median 13.5 years; HR 12.7, 95% CI 7.1-23.0), intermediate-1 (2-3 points; median 20.7 years; HR 3.8, 95% CI 2.3-6.4) and low (0-1 points; median 47 years). The AAA model (Akaike Information Criterion [AIC] 621) performed better than IPSET (AIC 647) and was subsequently validated by an external University of Florence ET cohort (N = 485). None of the AAA variables predicted LFS while ALC <1.7 × 109 /L was associated with inferior MFFS (p = .01). Adverse mutations (p < .01) and karyotype (p < .01) displayed additional prognostic value without disqualifying the prognostic integrity of the AAA model. This study proposes a simple and globally applicable survival model for ET, which can be used as a platform for further molecular refinement. This study also suggests a potential role for immune-related biomarkers, as a prognostic tool in myeloproliferative neoplasms.© 2023 The Authors. American Journal of Hematology published by Wiley Periodicals LLC.