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December 8, 2025Blood

Low hematologic event-free survival with age in shwachman-diamond syndrome

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Authors

JDJean DonadieuService de Santé des Armées XZXiaofan ZhuChinese Academy of Medical Sciences & Peking Union Medical College LFLucy C. FoxThe University of Melbourne

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Implication

Retrospective analysis reveals high cumulative risk of myeloid malignancies and transplant needs in Shwachman Diamond syndrome patients, indicating urgent need for early intervention.

Key Points

Severe hematologic complications are faced by 78% of patients with shwachman diamond syndrome by age 50.
The median survival time from diagnosis was 3.2 years for myelodysplastic syndrome and just 1 year for acute myeloid leukemia.
Cumulative risks for myeloid malignancies increased significantly with age, reaching 49.7% by age 50.
A comprehensive approach is essential for patients with SDS due to the high risk of severe bone marrow failure requiring hematopoietic stem cell transplant.

Cite This Study

Donadieu et al. (2025) studied this question.

synapsesocial.com/papers/69362f364fa91c937236d34b https://doi.org/10.1182/blood-2025-748

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Implication

Retrospective analysis reveals high cumulative risk of myeloid malignancies and transplant needs in Shwachman Diamond syndrome patients, indicating urgent need for early intervention.

Low hematologic event-free survival with age in shwachman-diamond syndrome

Discussion

Member takes

Implication

Key Points

Cite This Study

Low hematologic event-free survival with age in shwachman-diamond syndrome

Discussion

Member takes

Implication

Key Points

Cite This Study

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