Thromboembolism and pulmonary hypertension after splenectomy in thalassemia: a case report

Background: Pulmonary embolism (PE) and pulmonary hypertension (PH) are common respiratory diseases that require a thorough etiological workup. However, the pathophysiology of PE and PH in thalassemia patient post-splenectomy can be easily overlooked by both hematologists and pulmonologists. Case presentation: A 38-year-old man, who had diagnosed with α-thalassemia 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). Further accessory examination containing tumor markers, immunological indicators, thrombophilia screen and genetic testing could not elucidate potential provoking factors and pathogeny. Management consisted of curative anticoagulation, antibiotherapy, diuretics and hyperosmotic dehydration, leading to clinical improvement and subsequent stability on follow-up. Conclusion: The understanding of PE and PH following splenectomy in patients with thalassemia are often suboptimal, let alone the early management and treatment. It necessitates an integrated, multidisciplinary strategy involving hematology and pulmonology specialists.