Giant right ventricular outflow tract thrombus in hereditary spherocytosis: a case report

Abstract

BackgroundIn 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 presentationA 28 year-old smoking women who had previously undergone splenectomy due to hereditary spherocytosis with a moderate thrombocytosis (platelet count 553–635*109/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.ConclusionsThe 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.Electronic supplementary materialThe online version of this article (doi:10.1186/s12959-016-0083-3) contains supplementary material, which is available to authorized users.

Super-Selective Partial Splenic Embolization for Hereditary Spherocytosis in Children: A Single-Center Retrospective Study

“…The previous studies have shown that common complications of total splenectomy include postoperative infection, OPSI, surgical bleeding, injury of adjacent organs, and VTE (2,12,13). The most severe complications of PSE are abdominal infection and spleen abscess formation, which is deadly for patients in severe cases (14).…”

Super-selective Partial Splenic Embolization for Hereditary Spherocytosis in Children: A Single-Center retrospective Study

“…Most patients' symptoms resolve within 1 week after surgery. Previous studies have shown that common complications of total splenectomy include postoperative infection, OPSI, surgical bleeding, injury of adjacent organs and venous thromboembolism (VTE) 2,12,13 . The most serious complications of PSE are abdominal infection and spleen abscess formation, which is deadly for patients in severe cases 14 .…”

Coronary Embolism Presenting as NSTEMI in a Patient with Splenectomy

“…Few cases of thromboembolism have been reported with a link to hereditary spherocytosis (HS), particularly in patients with a prior history of splenectomy. 5 – 9 We present a case of a patient who was transferred from an outside hospital showing non-ST elevation myocardial infarction (NSTEMI) and who subsequently was diagnosed with CE.…”