Recurrent Pulmonary Embolism and Pulmonary Hypertension in a Patient with β-Thalassemia Intermedia

Patients with β-thalassemia intermedia are at increased risk of thromboembolic events and multifactorial pulmonary arterial hypertension. A pro-thrombotic state, including decreased levels of natural anticoagulant proteins and chronic platelet activation, has been shown in these patients, in particular after splenectomy. We report the case of a 54-year-old splenectomized β-thalassemic patient with a history of unprovoked deep venous (femoro-politeal) thrombosis, complicated by a pulmonary embolism event at the age of 37, recurrent episodes of superficial vein thrombosis of the lower limbs with leg ulcers, and a progressive severe pulmonary arterial hypertension, related to recurrences of pulmonary embolism, despite long-term, well conducted oral anticoagulant treatment (vitamin K antagonists and apixaban). After the performance of a right heart catheterization, pulmonary endarterectomy was not considered indicated in this patient because of the distal localization of thrombi. Aspirin treatment was added to vitamin K antagonists. Riociguat and ambrisentan therapy induced an improvement of both symptoms and echocardiographic picture over 12-mo follow-up. Splenectomized thalassemic patients are at high risk of thromboembolic events, and pulmonary hypertension, despite common, is yet a poorly understood complication. Clear recommendations on the management of such condition are lacking due to the limited data regarding the use of vasodilators, anticoagulants (vitamin K antagonists, heparins, direct oral anticoagulants) and antiplatelet agents.