Multiple littoral cell angioma of the spleen in a case of myelodysplastic syndrome

Abstract

Littoral cell angioma (LCA) of the spleen is a relatively recently described rare entity, which is associated with haemophagocytosis. In this case report, we will present a case of myelodysplastic syndrome with multiple LCA of the spleen. The patient presented with pancytopenia and multiple haemangiomas were observed in spleen during diagnostic abdominal ultrasound. She required a transfusion of one to two units of packed blood every 4-6 weeks and she eventually underwent a splenectomy in order to decrease the transfusion requirement. Although the patient's pancytopenia improved after surgery, the patient did not achieve a normal haemoglobin level in the two years following the splenectomy. Following further studies, bone marrow aspiration was undertaken and demonstrated minimal dysplasia in erythroid series including nuclear abnormalities, irregular cytoplasmic borders and irregular staining of the cytoplasm. The patient was diagnosed as myelodysplastic syndrome. As LCA of the spleen and myelodysplastic syndrome may both cause cytopenia their co-existence may complicate the clinical presentation leading to an incomplete or inappropriate diagnosis. In cases of haematological disorders with an atypical course, the radiological examination of the spleen should be considered and the possibility of angiomas must be included to the differential diagnosis.