J Korean Ophthalmol Soc.  2017 Jun;58(6):745-750. 10.3341/jkos.2017.58.6.745.

Retinoic Acid Syndrome and Pseudotumor Cerebri in Patients with Acute Promyelocytic Leukemia

• Kim JH
• Shin YT
• Park DH
• Kim IT
• Shin JP

• KMID: 2382687
• DOI: http://doi.org/10.3341/jkos.2017.58.6.745

Abstract

PURPOSE
To report cases of a retinal, preretinal and vitreous hemorrhage due to retinoic acid syndrome and a papilledema caused by increased intracranial pressure in patients with acute promyelocytic leukemia treated with all-trans retinoic acid (ATRA).
CASE SUMMARY
(Case 1) A 48-year-old female visited our clinic with headache, dyspnea, and visual disturbance during ATRA medications for acute promyelocytic leukemia. Her visual acuity of both eyes was hand motion, and fundus examination revealed extensive retinal, preretinal, and vitreous hemorrhage. The laboratory test showed leukocytosis and computed tomography of brain and chest revealed subarachnoid hemorrhage and pulmonary alveolar hemorrhage. She was diagnosed with retinoic acid syndrome and was treated with vitrectomy in both eyes. (Case 2) A 17-year-old male, who had been treated with ATRA for acute promyelocytic leukemia, had headache and visual disturbance. His best-corrected visual acuity was 1.0 in both eyes, but the fundus examination showed papilledema and retinal hemorrhage in both eyes. Goldmann visual field examination revealed an enlarged blind spot in both eyes. He was diagnosed with papilledema caused by ATRA induced increased intracranial pressure, and was observed.
CONCLUSIONS
Patients with acute promyelocytic leukemia treated with ATRA may have retinoic acid syndrome and increased intracranial pressure that could result in retinal hemorrhage, vitreous hemorrhage, and papilledema.

Keyword

Acute promyelocytic leukemia; All-trans retinoic acid; Papilledema; Pseudotumor cerebri; Retinoic acid syndrome

MeSH Terms

Adolescent
Brain
Dyspnea
Female
Hand
Headache
Hemorrhage
Humans
Intracranial Pressure
Leukemia, Promyelocytic, Acute*
Leukocytosis
Male
Middle Aged
Optic Disk
Papilledema
Pseudotumor Cerebri*
Retinal Hemorrhage
Retinaldehyde
Subarachnoid Hemorrhage
Thorax
Tretinoin*
Visual Acuity
Visual Fields
Vitrectomy
Vitreous Hemorrhage
Retinaldehyde
Tretinoin

Figure

• 

  Figure 1 Case 1. (A, B) Fundus photography showed extensive retinal, preretinal, and vitreous hemorrhage in both eyes. (C) The brain computed tomography showed subtle focal high density (red arrow) at left frontal lobe sulcus due to subarachnoid hemorrhage. (D) A contrast-enhanced chest computed tomography revealed patchy ground-glass opacity lesions (green arrow) in the right lower lobe suggesting pulmonary alveolar hemorrhage.

• 

  Figure 2 Six months after pars plana vitrectomy in case 1. (A, B) Fundus photography showed relatively stable appearance, and optical coherence tomography revealed foveal atrophy in both eyes.

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  Figure 3 Case 2. (A, B) Fundus photography showed papilledema, retinal hemorrhage, and preretinal hemorrhage. (C, D) Goldmann visual fields revealed enlarged blind spots in both eyes.

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  Figure 4 One month later in case 2. (A, B) Fundus photography showed that papilledema, retinal hemorrhage, and preretinal hemorrhage were diminished in both eyes.

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