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

Clinical caseA 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.DiscussionOptic 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.

ResumenCaso clínicoVarón 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.DiscusiónLa 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.