Shwachman-Diamond syndrome: The risk of AML is 20% by the age of 18 years; MECOM: The risk of myeloid malignancy is 5%; RUNX1: The lifetime risk of AML is 44%; CEBPA: It is seen that about 100% patients develop AML.28–30. The gene discussed is CEBPA; the disease is Shwachman-Diamond syndrome.