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Examine the survival and mortality trends of Erdheim-Chester disease over time in relation to targeted therapies.

December 8, 2025Blood

Evolving insights and changes in survival of erdheim-chester disease over time

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Retrospective cohort reveals increased mortality rates in Erdheim-Chester disease, suggesting targeted therapies may help.

Examine the survival and mortality trends of Erdheim-Chester disease over time in relation to targeted therapies.
Retrospective cohort study involving patients diagnosed with Erdheim-Chester disease between 2007 and 2024.
Survival analysis using Kaplan-Meier method and standardized mortality ratios derived from U.S. Census data.
Cox proportional hazards analysis to compare risk of death pre- and post-2015.
159 patients diagnosed with Erdheim-Chester disease; median follow-up of 4.0 years.
Overall survival rate at 5 years was 80%, with higher mortality compared to expected rates (SMR of 2.5).
No significant reduction in mortality post-2015, with a hazard ratio of 0.59 for diagnosis after 2015.

Poleksic et al. (2025) studied this question.

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