Extensive systemic thromboembolism in a patient with α-thalassemia after splenectomy: a case report

Abstract

Pulmonary embolism (PE) is a common respiratory disease that require a thorough etiological workup. However, the pathophysiology of PE in thalassemia patient post-splenectomy can be easily overlooked by pulmonologists. A 38-year-old man, who had been diagnosed with α-thalassemia intermedia at 7 months of age, and subsequently underwent splenectomy nine years ago due to severe anemia, developed chest tightness and shortness of breath, and progressed to dyspnea at rest accompanied by dizziness and abdominal pain. Chest and abdomen enhanced CT scan revealed extensive venous thrombosis including pulmonary artery, portal vein, mesenteric vein, hepatic vein and possibly cerebral thrombosis. Cardiac echocardiography demonstrated severe PH (tricuspid-valve regurgitant jet velocity was 4.2 m/s). Investigations including tumor markers, immunological indicators, thrombophilia screen and genetic testing did not elucidate potential provoking factors and pathogeny. Management consisted of curative anticoagulation, antibiotics, diuretics and hyperosmotic dehydration, leading to clinical improvement and subsequent stability on follow-up. It is important to recognize PE post splenectomy in patients with thalassemia. It necessitates an integrated, multidisciplinary strategy involving hematology and pulmonology specialists.