A 69-year-old man with a personal history of M1 AML (myeloblastic without differentiation), in complete remission (CR) for 11 months after chemotherapy treatment according to the PETHEMA-LAM 99 protocol. In February 2008 he was admitted for catarrhal respiratory symptoms, with severe nasal congestion, with no response to antibiotics. He also presented erythematous papules on his back, supraclavicular and submaxillary adenopathies.
The patient was referred by the haematologist for presenting red eye of two days' evolution, with no other ocular symptoms. On examination he had a corrected visual acuity of 20/20 in OD and 18/20 in OI. Ocular reflexes, motility and tonometry were normal. Anterior pole biomicroscopy showed a salmon-coloured raised fleshy lesion on the upper and lower tarsus in OD. In the OI, a similar lesion was found in the inferior tarsal conjunctiva. No alterations were observed in the fundus.

The possibility of leukaemic involvement of the conjunctiva was considered and a biopsy of the lesion was performed. Histology showed a diffuse proliferation of myeloid cells arranged under the conjunctival epithelium, which were immunohistochemically positive for myeloperoxidase and CD43, negative for B and T cell markers (CD20, CD3, CD5), as well as for TdT, CD34 and CD117, and the case was diagnosed as a granulocytic sarcoma.

New biopsies confirmed the involvement of the nasal mucosa and skin. Based on these findings, the diagnosis was SM affecting conjunctiva, skin and nasal mucosa. However, no relapse was observed in peripheral blood and bone marrow smears, so it was considered that CR persisted at this level. Lumbar puncture and all other examinations were normal.
Following the diagnosis of extramedullary recurrence in the form of SM of AML, second-line treatment was started according to the FLAG-IDA protocol with a good response of the infiltrative lesions. The patient died in May 2008, three months after the diagnosis of SM, due to multi-organ failure.