Optic nerve swelling as seen in recurrent leukemia



A 10-year old Hispanic boy was diagnosed with T-cell acute lymphoblastic leukemia at age 7 years, with initial bone marrow involvement and without central nervous system (CNS) involvement. He was treated successfully with chemotherapy and completed treatment 2.5 years later. At age 13 years, he sustained a relapse, with bone marrow involvement and again there was no CNS involvement.

He successfully completed 2 years of treatment with chemotherapy. Seven months later, while off therapy at age 15 years, he complained to his oncologist at a routine check-up about blurry vision and pain in the left eye. On examination by the neuro-ophthalmologist (M.R.A.), he was found to have visual acuity of 20/20 in the right eye and 20/100 in the left eye, with severe optic nerve edema (Figure 1 and 2).

Cerebral spinal fluid showed 20 nucleated cells/mm3, all blasts. His bone marrow showed relapse, and he had new testicular involvement. The patient was treated with chemotherapy, including intrathecal chemotherapy.

He went into marrow remission, and his CNS cleared of blast cells, but there was no improvement of his visual acuity. The optic neuropathy was the first clinical sign of his recurrence. All visual complaints in patients with leukemia should be evaluated promptly. Leukemia can affect the uvea, retina, optic disc, vitreous, or extraocular tissues. This case presented with optic nerve edema, which can occur at any time during the course of the disease and is associated with CNS involvement. The edema may result from meningeal involvement, intracranial hypertension from medication, or local infiltration. Abnormal eye findings warrant an evaluation, including a complete blood count with differential, bone marrow examination, complete systemic staging, lumbar puncture for CSF cytology, and imaging studies.

Treatment is usually a combination of systemic chemotherapy appropriate to the type of leukemia and radiation therapy to the involved eye or eyes. The prognosis for visual recovery with optic disc involvement is poor in most cases. Madhu R. Agarwal, MD Pamela Wagner, BS, MS Antranik A. Bedros, MD

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