Some ITs are associated with extra-hematological manifestations such as sensorineural deafness (MYH9-related disease, DIAPH1-related disease) or myopathy (Storkmorken syndrome) whilst others have a predisposition to hematological malignancies such as acute myeloid leukemia (AML), acute lymphoblastic leukemia (ALL), chronic myeloid leukemia (CML), and myelodysplastic syndrome (MDS) [1–4]. The gene discussed is MYH9; the disease is acute myeloid leukemia.