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. 2022 Feb 14:13:839081.
doi: 10.3389/fneur.2022.839081. eCollection 2022.

Erythrocyte Indices in Creutzfeldt-Jakob Disease Predict Survival Time

Yu Kong  1 , Zhongyun Chen  1 , Jing Zhang  1 , Liyong Wu  1
Affiliations

Erythrocyte Indices in Creutzfeldt-Jakob Disease Predict Survival Time

Yu Kong et al. Front Neurol. .

Abstract

Background: Creutzfeldt-Jakob disease (CJD) is a devastating neurodegenerative disease caused by propagation of abnormally folded prion proteins (PrPSc). Some fluid biomarkers have been reported to be associated with disease duration in CJD. Based on studies which have found that prion protein (PrPC) played a role in erythrocytic hematopoiesis, we evaluated the association between peripheral red blood cell indices and survival time in CJD.

Methods: 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.

Results: 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, p = 0.012) and HCT (hazard ratio 1.689, 95% CI 1.112-2.565, p=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.

Conclusions: 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.

Keywords: Creutzfeldt-Jakob disease; erythrocyte indices; hematocrit; hemoglobin; red cell indices; survival time.

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Conflict of interest statement

The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.

Figures

Figure 1
Figure 1
Flow diagram of patient enrollment.
Figure 2
Figure 2
Association between survival time and red cell indices. Hb (A) and HCT (B) levels in patients with CJD are plotted against survival time, and were analyzed using Spearman's rho correlation analysis.
Figure 3
Figure 3
Survival analyses of CJD patients with high vs. low HCT (A) and Hb (B) levels. A median split was conducted on HCT (41.10%) and Hb (140.00 g/l) levels. Higher baseline Hb and HCT levels were associated with a shorter survival time.
See this image and copyright information in PMC

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References

    1. Takada LT, Geschwind MD. Prion diseases. Semin Neurol. (2013) 33:348–56. 10.1055/s-0033-1359314 - DOI - PubMed
    1. Johnson DY, Dunkelberger DL, Henry M, Haman A, Greicius MD, Wong K, et al. . Sporadic Jakob-Creutzfeldt disease presenting as primary progressive aphasia. JAMA Neurol. (2013) 70:254–7. 10.1001/2013.jamaneurol.139 - DOI - PMC - PubMed
    1. Pocchiari M, Puopolo M, Croes EA, Budka H, Gelpi E, Collins S, et al. . Predictors of survival in sporadic Creutzfeldt-Jakob disease and other human transmissible spongiform encephalopathies. Brain. (2004) 127(Pt 10):2348–59. 10.1093/brain/awh249 - DOI - PubMed
    1. Puoti G, Bizzi A, Forloni G, Safar JG, Tagliavini F, Gambetti P. Sporadic human prion diseases: molecular insights and diagnosis. Lancet Neurol. (2012) 11:618–28. 10.1016/S1474-4422(12)70063-7 - DOI - PubMed
    1. Llorens F, Rübsamen N, Hermann P, Schmitz M, Villar-Piqué A, Goebel S, et al. . A prognostic model for overall survival in sporadic Creutzfeldt-Jakob disease. Alzheimers Dement. (2020) 16:1438–47. 10.1002/alz.12133 - DOI - PubMed