Background:
In hereditary spherocytosis with severe anemia, splenectomy is a recommended treatment. However, the spleen carries an important role both in immune function and coagulation. The increased risk of bacterial infections associated with splenectomy is well known. Recently, hypercoagulation disorders have also been linked to splenectomy through loss of regulation of platelet activity, loss of filtering function and post-splenectomy thrombocytosis.
Case presentation:
A 28 year-old smoking women who had previously undergone splenectomy due to hereditary spherocytosis with a moderate thrombocytosis (platelet count 553-635*10(9)/L), presented with recurrent episodes of pulmonary embolisms. Further examination by multimodality cardiac imaging demonstrated a giant chronic thrombus in the right ventricular outflow tract, which eventually had to be surgically removed.
Conclusions:
The present case highlights the increased risk of severe thromboembolic complications following therapeutic splenectomy in hereditary spherocytosis, and emphasis the important role of multimodality cardiac imaging in recurrent pulmonary embolism, diagnosing a giant chronic thrombus in the right ventricular outflow tract.