Diagnosis of Acute Promyelocytic Leukemia After Presentation to Neuro-Ophthalmology

J Neuroophthalmol. 2022 Mar 1;42(1):e181-e186. doi: 10.1097/WNO.0000000000001259. Epub 2021 Aug 25.


Background: Acute promyelocytic leukemia (APML) is a medical emergency that can initially present with neuro-ophthalmologic signs. Early recognition is crucial, and immediate treatment with all-trans retinoic acid (ATRA) and arsenic trioxide (ATO) can be life-saving. The goal of this study was to describe patients who first presented to neuro-ophthalmology and were subsequently diagnosed with APML.

Methods: We retrospectively reviewed consecutive patients seen at a tertiary neuro-ophthalmology centre. Patients with an unknown diagnosis of APML at presentation who subsequently went on to receive this diagnosis were included. Clinical characteristics, neuro-ophthalmologic findings, and outcome were retrieved.

Results: A total of 3 patients (2 women and 1 men) with a mean age of 30.7 (range 24-33) years were included in the study. Neuro-ophthalmologic diagnoses at presentation were severe hemorrhagic papilledema related to dural venous sinus thrombosis, hemorrhagic bilateral optic disc edema, and left homonymous hemianopia related to an occipital lobe hemorrhage. At diagnosis, the average hemoglobin was 83.7g/L (range 78-104), and the platelet count was 39.3 × 109/L (range 15-77). All patients were treated with ATRA and ATO. One patient developed papilledema and sixth nerve palsies related to this treatment, which resolved with acetazolamide. Clinical follow-up ranged from 6 to 12 months, and all patients were in clinical remission about systemic APML.

Conclusion: Neuro-ophthalmologic symptoms may be the first manifestations of APML, and a complete blood count is an essential test in patients presenting with optic disc edema, especially if hemorrhagic.

MeSH terms

  • Adult
  • Antineoplastic Combined Chemotherapy Protocols / adverse effects
  • Arsenic Trioxide / therapeutic use
  • Female
  • Humans
  • Leukemia, Promyelocytic, Acute* / complications
  • Leukemia, Promyelocytic, Acute* / diagnosis
  • Leukemia, Promyelocytic, Acute* / drug therapy
  • Male
  • Ophthalmology*
  • Papilledema* / etiology
  • Retrospective Studies
  • Treatment Outcome
  • Tretinoin / therapeutic use
  • Young Adult


  • Tretinoin
  • Arsenic Trioxide