1. Therapy-related Myeloid Neoplasms in Children: A Single-institute Study
- Author
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Li, Geling, Holly, Taylor, Kelly, David R., Reddy, Vishnu, Mikhail, Fady M., Carroll, Andrew J., and Kutny, Matthew A.
- Abstract
Therapy-related myeloid neoplasm (t-MN) in the pediatric population is not well characterized. We studied 12 pediatric patients diagnosed with t-MN in our institution since 2006. The median age at the t-MN diagnoses was 14.8 years (range, 9 to 20 y). The primary malignancies included 9 solid tumors and 3 hematopoietic malignancies. Rhabdomyosarcoma (n=4) was the most common primary malignancy. Five of the 9 patients with solid tumors and all 3 patients with hematopoietic malignancies had primary neoplasms involving bone marrow. The median latency period was 5.2 years (range, 1.8 to 13.8 y). Thrombocytopenia was present in all patients at the t-MN diagnoses. Complete or partial monosomy of chromosome 5 or 7 were the 2 most common cytogenetic abnormalities. A quarter of patients demonstrated a genetic predisposition to t-MN: 1 with Li-Fraumeni syndrome with a germline TP53R248Q mutation, 1 with Noonan syndrome with a somatic mutation (PTPN11S502T), and 1 with a constitutive chromosomal translocation [t(X;9)(p22;q34)] and a germline TP53L130V mutation. Outcomes remain poor. Two patients survived 3 and 5.1 years after hematopoietic stem cell transplantation.
- Published
- 2022
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