Patient aged 66 years, was admitted for splenomegaly grade III/IV, haematological hypersplenism and medium / large secondary anemia, diagnosed in the hematology clinic, where a myeloproliferative syndrome was denied. Was performed splenectomy, splenic lodge toilet and drainage. Histopathology was established the diagnosis of splenic sarcoidosis. Good postoperative evolution. Main issues discussed are etiopathogeny, diagnostic and therapeutic, that this rare disease of unknown etiology is giving.
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