Persistent thrombocytopaenia in a young man with splenomegaly, rebound thrombocytosis after splenectomy and subsequent pulmonary embolism: splenic littoral cell angioma and associated events

Abstract

Littoral cell angioma (LCA) is a rare endothelial cell neoplasm in the spleen. Although many cases of LCA are asymptomatic, some present with signs and symptoms related to splenomegaly, whereas others manifest with haematological abnormalities, including anaemia and/or thrombocytopaenia (ie, hypersplenism). We report a case of LCA presenting with chronic thrombocytopaenia, probably due to splenic sequestration of platelets or phagocytosis of platelets by neoplastic cells. Following therapeutic splenectomy, the patient suffered from a marked rebound thrombocytosis and subsequently developed pulmonary embolisms. He was treated with anticoagulant therapy combined with antiplatelet therapy, and his symptoms were quickly resolved. This case emphasises an exclusion of primary splenic disorders in patients with chronic thrombocytopaenia, especially in those with splenomegaly and the contemplation of thromboembolism prophylaxis postsplenectomy.