Key Points: MYH-9-Related Disorders

MYH9-related disorders are a group of autosomal dominant conditions characterized by inherited thrombocytopenia and platelet macrocytosis due to variants in the MYH9 gene. Thrombocytopenia represents the primary antepartum concern and requires close monitoring during pregnancy. Patients are at increased risk of bleeding, including postpartum hemorrhage, although reported risk varies across studies. Management during pregnancy and delivery is primarily supportive, with platelet transfusion used when clinically indicated; thrombopoietin receptor agonists remain investigational and have not been studied in pregnancy.