Sclerosing angiomatoid nodular transformation of spleen is a rare, benign vascular lesion with an uncertain pathogenesis. It has been described as a separate entity through specific histopathological characters. It is usually asymptomatic, occurring commonly in adult females. Only a few cases of paediatric cases have been reported which have been commonly symptomatic. This disease has excellent prognosis after splenectomy, which is the only treatment. We report the case of an eight-year-old girl who presented with distended abdomen and history of bleeding from the nose following a road traffic accident. Examination revealed stunted height, decreased weight, tachypnoea, tachycardia, anaemia and a firm, massive spleen. Lab investigations further revealed microcytic anaemia, thrombocytopenia, deranged platelet profile and low vitamin B12 and folate levels. Computed tomography confirmed enlarged spleen. Therefore, a diagnostic biopsy was planned which confirmed sclerosing angiomatoid nodular transformation of spleen. Splenectomy was successfully performed soon after and the child is now healthy with no remissions of previous symptoms.
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