Ocular palsy associated with aggressive NK-cell leukemia

Abstract

A 22-year-old woman was referred to our hospital with a 1-month history of recurrent fever, subacute onset of bilateral orbital pain, and diplopia. Physical examination revealed the limits of supraduction of her right eye and infraduction of her left eye. A bone marrow aspirate from the ilium showed scattered large immature-looking lymphocytes (15.8%) with pale cytoplasm, fine nuclear chromatin, and nucleoli (Fig. 1). These cells were stained positively with CD56, TIA-1, granzyme, and perforin, and were stained negatively with CD3. In situ hybridization for EBER demonstrated that the cells were EBV-positive. A diagnosis of aggressive NK-cell leukemia was made. Magnetic resonance imaging of orbital cavity showed marked enlargement, and diffuse high intensity signals in the right superior rectus muscle and left inferior rectus muscle on fat-saturated T2-weighted image (Fig. 1). These lesions were diffusely contrast-enhanced on fat-saturated T1-weighted image (Fig. 2). After two courses of dexamethasone, methotrexate, ifosfamide, L-asparaginase, and etoposide chemotherapy,