Pediatric Myeloid neoplasms may have a predisposing germline mutation, with the percentage of tumors with germline predisposition varying depending on the type of myeloid neoplasm. For instance, approximately 25% of JMML cases have germline mutations in NF1 or CBL genes, while around 7% of pediatric AML cases have germline gene mutations in CEBPA, RUNX1, GATA2, SAMD9/SAMD9L, ANKRD26, ELANE, HAX1, NF1, Shwachman-Diamond syndrome-related genes, Fanconi anemia-related genes, or Dyskeratosis congenita-related genes.^1,2 Additionally, Down syndrome patients are at an increased risk of developing both transient abnormal myelopoiesis (TAM) and full-scale AML. Approximately 25% of Down syndrome patients may have TAM, although more than half of these cases are asymptomatic. Furthermore, 1.4% of Down syndrome patients may develop AML by age 4.³ Interestingly, patients with myeloid neoplasms with germline predisposition typically have a worse prognosis than those with sporadic myeloid neoplasms, but there are some exceptions. For example, Down syndrome-related AML, JMML with CBL syndrome, MDS with GATA2, and AML with CEBPA may have more favorable outcomes.