Therapy-related Myeloid Neoplasms in Children: A Single-institute Study
| Autor: | David R. Kelly, Geling Li, Taylor Holly, Andrew J. Carroll, Vishnu Reddy, Fady M. Mikhail, Matthew A. Kutny |
|---|---|
| Rok vydání: | 2021 |
| Předmět: |
Adult
Male Oncology medicine.medical_specialty Monosomy Myeloid Adolescent medicine.medical_treatment Hematopoietic stem cell transplantation Myeloid Neoplasm Li-Fraumeni Syndrome Young Adult Germline mutation Internal medicine Rhabdomyosarcoma Humans Medicine Genetic Predisposition to Disease Child Myeloproliferative Disorders business.industry Noonan Syndrome Hematopoietic Stem Cell Transplantation Infant Neoplasms Second Primary Hematology Allografts medicine.disease PTPN11 medicine.anatomical_structure Child Preschool Hematologic Neoplasms Pediatrics Perinatology and Child Health Chromosomes Human Pair 5 Noonan syndrome Female Chromosome Deletion business Chromosomes Human Pair 7 |
| Zdroj: | Journal of Pediatric Hematology/Oncology. 44:e109-e113 |
| ISSN: | 1077-4114 |
| DOI: | 10.1097/mph.0000000000002097 |
| Popis: | 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 TP53 R248Q mutation, 1 with Noonan syndrome with a somatic mutation (PTPN11 S502T), and 1 with a constitutive chromosomal translocation [t(X;9)(p22;q34)] and a germline TP53 L130V mutation. Outcomes remain poor. Two patients survived 3 and 5.1 years after hematopoietic stem cell transplantation. |
| Databáze: | OpenAIRE |
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