A 76 years old man, followed since 1999 because of homogeneous echographic splenomegaly. Hb levels were normal (13.2 g/dl) with 50000 platelets/ml and 3900 leucocytes (75% PMN). A bone marrow examination disclosed hypercellularity. As ITP could not be ruled out, danazol 400 mg/day was started and maintained during 3 months without any increase of platelet levels. His platelet counts remained between 45000 and 80000 during follow-up. In February 2006, a heterogeneous spleen was seen during an abdominal ultrasound and confirmed with a CT scan. A new bone marrow examination was slightly hyper cellular for his age and no abnormal lymphoid populations were found by FACS or immunohistochemistry. Splenectomy was performed in June 2006. Post op platelet levels increased to 449.000. Spleen was slightly enlarged, with reddish surface.