JCO precision oncology · 2025
Hematologic Malignancy Frequency, Phenotypes, and Outcomes in Li-Fraumeni Syndrome.
СтатьяНазвание и аннотация: Перевод в очереди
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Vagher Jennie, Zakas Anna, Donovan Lauren, Shoger Kyle, Naumer Anne, Bly Journey, Smith-Simmer Kelcy, Maese Luke, Schiffman Joshua, Kohlmann Wendy, Garzon Ramiro, Churpek Jane E, Osman Afaf E G
Аннотация
PURPOSE: Hematologic malignancies (HMs) account for 4%-10% of cancers in individuals with Li-Fraumeni syndrome (LFS), but their phenotypic spectrum and clinical outcomes remain incompletely characterized.
METHODS: We conducted a retrospective cohort study at The University of Utah and University of Wisconsin-Madison. Cancer genetics registries were reviewed to identify all unrelated families with LFS seen between 2010 and the present with at least one individual with a pathologically confirmed HM. A literature review was conducted to identify individuals in the published literature with LFS with an HM. HM molecular characteristics, treatment, and outcomes were recorded.
RESULTS: Among 121 total families with LFS at our institutions, 17 patients from 16 (13%) families diagnosed with LFS had an HM. A literature review found an additional 83 patients with detailed descriptions, for a total of 99 patients with LFS and an HM. The spectrum of HMs included 10 subtypes with a propensity for lymphoid over myeloid diagnoses. Most HMs did not occur after cytotoxic therapy and often responded to usual sporadic HM regimens, but in a subset, unusual toxicities were encountered, especially after hematopoietic stem-cell transplantation.
CONCLUSION: Our large cohort of patients with HM and LFS suggest a broad spectrum of HMs in LFS with more lymphoid than myeloid, more de novo than postcytotoxic therapy, and more favorable outcomes than previous reports.
METHODS: We conducted a retrospective cohort study at The University of Utah and University of Wisconsin-Madison. Cancer genetics registries were reviewed to identify all unrelated families with LFS seen between 2010 and the present with at least one individual with a pathologically confirmed HM. A literature review was conducted to identify individuals in the published literature with LFS with an HM. HM molecular characteristics, treatment, and outcomes were recorded.
RESULTS: Among 121 total families with LFS at our institutions, 17 patients from 16 (13%) families diagnosed with LFS had an HM. A literature review found an additional 83 patients with detailed descriptions, for a total of 99 patients with LFS and an HM. The spectrum of HMs included 10 subtypes with a propensity for lymphoid over myeloid diagnoses. Most HMs did not occur after cytotoxic therapy and often responded to usual sporadic HM regimens, but in a subset, unusual toxicities were encountered, especially after hematopoietic stem-cell transplantation.
CONCLUSION: Our large cohort of patients with HM and LFS suggest a broad spectrum of HMs in LFS with more lymphoid than myeloid, more de novo than postcytotoxic therapy, and more favorable outcomes than previous reports.