Unilateral optic disk edema with central retinal artery and vein occlusions as the presenting signs of relapse in acute lymphoblastic leukemia

Abstract

Clinical case

A 39-year-old man with Philadelphia chromosome-positive acute lymphoblastic leukemia (LAL Ph+) developed progressive vision loss to no light perception in his right eye. He had optic disk edema and later developed central artery and vein occlusions. Pan-photocoagulation and radiotherapy of the whole brain were performed in several fractions. Unfortunately the patient died of hematological relapse 4 months later.

Discussion

Optic nerve infiltration may appear as an isolated sign of a leukemia relapse, even before a hematological relapse occurs. Leukemic optic neuropathy is a critical sign, not only for vision, but also for life, and radiotherapy should be immediately performed before irreversible optic nerve damage occurs.

Resumen

Caso clínico

Varón con amaurosis en ojo derecho y diagnóstico previo de leucemia linfoblástica aguda con cromosoma Philadelphia positivo (LAL Ph+). Presenta edema sectorial del disco óptico y, posteriormente, obstrucción de arteria y vena central de la retina. Se realiza panfotocoagulación retiniana y tratamiento radioterápico holocraneal. A los 4 meses sufre recaída hematológica, siendo finalmente exitus.

Discusión

La afectación del nervio óptico puede presentarse como único hallazgo en una recurrencia de una LAL, precediendo incluso a la recaída hematológica. Constituye por tanto una urgencia no solo visual sino, sobre todo, vital, que precisa de un tratamiento intensivo muchas veces complementado con radioterapia.