HIGHLIGHTS
SUMMARY
Therapy in a Child with Inherited thrombocytopenias (ITs) represent a heterogeneous group of diseases characterized by a congenital reduction in platelet number that could lead to a wide severity in bleeding symptoms. Patients with ITs sometimes need platelet transfusions before surgical interventions or invasive procedures because their platelet count is below the safe threshold for the specific procedures. MYH9-related diseases (MYH9-RD) are characterized by enlarged platelets (more than 40% of >3.9 µm) and congenital thrombocytopenia (platelet count less than 150 × 109 /L). Thrombopoietin receptor agonists (TPO-RAs), eltrombopag and romiplostim, are . . .
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