LA-MARRVEL: A Knowledge-Grounded, Language-Aware LLM Framework for Clinically Robust Rare Disease Gene Prioritization

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Main Authors: Lee, Jaeyeon, Yao, Lin, Jeong, Hyun-Hwan, Liu, Zhandong
Format: Preprint
Published: 2025
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author Lee, Jaeyeon
Yao, Lin
Jeong, Hyun-Hwan
Liu, Zhandong
author_facet Lee, Jaeyeon
Yao, Lin
Jeong, Hyun-Hwan
Liu, Zhandong
contents Rare disease diagnosis requires matching variant-bearing genes to complex patient phenotypes across large and heterogeneous evidence sources. This process remains time-intensive in current clinical interpretation pipelines. To overcome these limitations, We present LA-MARRVEL, a knowledge-grounded, language-aware LLM framework and designed for clinical robustness and practical deployment. LA-MARRVEL delivers a 12-15 percentage-point absolute improvement in Recall@1 over established gene prioritization approaches, showing that architectural design can drive substantial accuracy gains. We found that the central contributor is structured, phenotype-rich prompt construction that explicitly encodes patient and disease phenotypes, preserving clinically meaningful context more effectively than disease labels alone. Across three real-world cohorts, LA-MARRVEL consistently improves gene-ranking performance, including in challenging cases where the causal gene was initially ranked lower by first-stage prioritization. For each candidate gene, the system delivers clinically relevant, ACMG-aligned reasoning that integrates phenotype concordance, inheritance patterns, and variant-level evidence into auditable explanations, enabling streamlined clinical review. These findings suggest that knowledge-grounded LLM layer can enhance existing rare-disease gene prioritization workflows without altering established diagnostic pipelines.
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publishDate 2025
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spellingShingle LA-MARRVEL: A Knowledge-Grounded, Language-Aware LLM Framework for Clinically Robust Rare Disease Gene Prioritization
Lee, Jaeyeon
Yao, Lin
Jeong, Hyun-Hwan
Liu, Zhandong
Genomics
Artificial Intelligence
Rare disease diagnosis requires matching variant-bearing genes to complex patient phenotypes across large and heterogeneous evidence sources. This process remains time-intensive in current clinical interpretation pipelines. To overcome these limitations, We present LA-MARRVEL, a knowledge-grounded, language-aware LLM framework and designed for clinical robustness and practical deployment. LA-MARRVEL delivers a 12-15 percentage-point absolute improvement in Recall@1 over established gene prioritization approaches, showing that architectural design can drive substantial accuracy gains. We found that the central contributor is structured, phenotype-rich prompt construction that explicitly encodes patient and disease phenotypes, preserving clinically meaningful context more effectively than disease labels alone. Across three real-world cohorts, LA-MARRVEL consistently improves gene-ranking performance, including in challenging cases where the causal gene was initially ranked lower by first-stage prioritization. For each candidate gene, the system delivers clinically relevant, ACMG-aligned reasoning that integrates phenotype concordance, inheritance patterns, and variant-level evidence into auditable explanations, enabling streamlined clinical review. These findings suggest that knowledge-grounded LLM layer can enhance existing rare-disease gene prioritization workflows without altering established diagnostic pipelines.
title LA-MARRVEL: A Knowledge-Grounded, Language-Aware LLM Framework for Clinically Robust Rare Disease Gene Prioritization
topic Genomics
Artificial Intelligence
url https://arxiv.org/abs/2511.02263