Non‐Tropical Idiopathic Splenomegaly (Dacie's Syndrome)

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Abstract
Five patients aged 22-69 yr with massive splenomegaly of unknown origin and features of hypersplenism are described. Splenectomy corrected the cytopenia(s) and abolished the symptoms in each case. The histological features of the spleen were nonspecific and included congestive changes and lymphoid hyperplasia. Of these patients, 2 developed non-Hodgkin''s lymphoma (NHL) 2 and 6 yr after splenectomy. A total of 46 cases of splenomegaly of unknown origin have been reported from UK, USA and Australia so far, and in 9, NHL developed 8-80 mo. after splenectomy. The questions relating to the pathogenesis of splenomegaly and the subsequent development of NHL remain to be answered. It was proposed that this entity be known as Dacie''s syndrome, after Sir John Dacie, who characterized it in 1969.