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ICARE Newsletter Summer 2018

Inherited Leukemias: The Importance of TP53/Li-Fraumeni Syndrome and Other Genes

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It has long been established that the risk for developing leukemia in childhood is high among individuals with Li-Fraumeni Syndrome; however, better understanding the characteristics of leukemia among these individuals is important to guide treatment approaches. In a study of children with Acute Lymphocytic Leukemia (ALL), those with a germline TP53 mutation (compared to those without a mutation) were older (median age of 15.5 years, compared to 7.3 years), were at a much higher risk of second cancers (25.1% versus 0.7%), and were more likely to have hypodiploid ALL (65.4% versus 1.2%), with poorer outcomes.1 This information may be important to guide treatment among these individuals, including type and timing of treatment.

In addition to Li-Fraumeni Syndrome, there are several other conditions that are associated with inherited susceptibility to leukemia among individuals of all ages, including those associated with bone marrow failure syndromes, those in which myelodysplastic syndrome is seen before the onset of leukemia, and those with primarily a leukemia risk.2 Patients with inherited hematologic malignancy syndromes may present without classic clinical signs of a particular familial syndrome or even a family history.3 As more patients with inherited forms are diagnosed, there remains a need for developing evidence-based recommendations because current recommendations are primarily based on expert consensus.4

1Qian M, et al. J Clin Oncol. 2018 Feb 20. PMID: 29300620.
2McReynolds LJ, et al. Hematology Am Soc Hematol Educ Program. 2017 Dec 8. PMID:29222262.
3Furutani et al. J Clin Oncol. 2017 Mar 20. PMID: 28297620.
4Godley LA, et al. Blood. 2017 Jul 27. PMID: 28600339.

Permanent link to this article: https://inheritedcancer.net/11nls2018/