Kong Yu, Chen Zhongyun, Zhang Jing, Wu Liyong
Department of Neurology, Xuanwu Hospital, Capital Medical University, Beijing, China.
Front Neurol. 2022 Feb 14;13:839081. doi: 10.3389/fneur.2022.839081. eCollection 2022.
Creutzfeldt-Jakob disease (CJD) is a devastating neurodegenerative disease caused by propagation of abnormally folded prion proteins (PrP). Some fluid biomarkers have been reported to be associated with disease duration in CJD. Based on studies which have found that prion protein (PrP) played a role in erythrocytic hematopoiesis, we evaluated the association between peripheral red blood cell indices and survival time in CJD.
We retrospectively collected data on peripheral red blood cell indices, including red blood cell (RBC) count, hemoglobin (Hb), hematocrit (HCT), mean corpuscular volume (MCV), mean corpuscular hemoglobin (MCH), mean corpuscular hemoglobin concentration (MCHC), and red cell distribution width (RDW), from 125 CJD patients. Cox proportional hazard models were generated to determine whether red cell indices correlated with survival time of patients with CJD.
Of the 125 included participants, 70 (56%) were male, and the mean age at diagnosis (SD) was 60.3 (9.5) years. Hemoglobin levels (hazard ratio 1.710, 95% CI 1.124-2.600, = 0.012) and HCT (hazard ratio 1.689, 95% CI 1.112-2.565, =0.014) were significantly associated with survival time after controlling for sex, age, and Barthel Index. Red blood cell count, MCV, MCH, MCHC, and RDW were not associated with survival time before or after adjusting for covariates.
Our study found that Hb and HCT were significantly associated with survival time in patients with CJD. These results may inform evaluation of the mechanisms of interaction between prion disease and hematopoiesis, and indicate that Hb and HCT may be potential prognostic biomarkers.
克雅氏病（CJD）是一种由异常折叠的朊病毒蛋白（PrP）传播引起的毁灭性神经退行性疾病。据报道，一些体液生物标志物与CJD的病程有关。基于发现朊病毒蛋白（PrP）在红细胞造血中起作用的研究，我们评估了外周血红细胞指标与CJD患者生存时间之间的关联。
我们回顾性收集了125例CJD患者的外周血红细胞指标数据，包括红细胞（RBC）计数、血红蛋白（Hb）、血细胞比容（HCT）、平均红细胞体积（MCV）、平均红细胞血红蛋白含量（MCH）、平均红细胞血红蛋白浓度（MCHC）和红细胞分布宽度（RDW）。生成Cox比例风险模型以确定红细胞指标是否与CJD患者的生存时间相关。
在125名纳入的参与者中，70名（56%）为男性，诊断时的平均年龄（标准差）为60.3（9.5）岁。在控制性别、年龄和巴氏指数后，血红蛋白水平（风险比1.710，95%置信区间1.124 - 2.600，P = 0.012）和HCT（风险比1.689，95%置信区间1.112 - 2.565，P = 0.014）与生存时间显著相关。在调整协变量之前或之后，红细胞计数、MCV、MCH、MCHC和RDW与生存时间均无关联。
我们的研究发现，Hb和HCT与CJD患者的生存时间显著相关。这些结果可能有助于评估朊病毒病与造血之间相互作用的机制，并表明Hb和HCT可能是潜在的预后生物标志物。
