Congenital Hemophilia‐A With High‐Titer Inhibitor and Essential Thrombocythemia: A Rare Coexistence

Background: The coexistence of congenital haemophilia A with a high-titre factor VIII inhibitor and essential thrombocythaemia (ET) represents a rare and complex clinical condition, combining a high bleeding risk with an increased thrombotic risk. Case: We report a patient with congenital haemophilia A and a persistent high-titre FVIII inhibitor who was subsequently diagnosed with JAK2-positive ET. The clinical management was further complicated by chronic hepatitis C and the development of a femoral haemophilic pseudotumour. Emicizumab was initiated to improve bleeding control, while hydroxyurea was used to reduce the thrombotic risk associated with ET. Conclusion: This case highlights the therapeutic challenges of simultaneously managing bleeding and thrombotic risks in patients with haemophilia and myeloproliferative neoplasms. An individualized, multidisciplinary approach is essential to achieve an appropriate haemostatic balance.