Acute macular neuroretinopathy associated with acute promyelocytic leukemia



To describe the first reported case of acute macular neuroretinopathy (AMN) associated with acute promyelocytic leukemia in a young Asian-Indian male.


We review the clinical and multimodal imaging findings in our patient that are characteristic of AMN.

Conclusions and importance

Ophthalmologists should be aware of the association of leukemia with AMN and consider hematologic work-up when assessing patients with AMN without the prototypical history or risk factors.


Acute macular neuroretinopathy (AMN), is a relatively rare retinal disease that was first described by Bos and Deutman in 1975 in healthy young females taking oral contraceptives. Affected patients classically present with acute paracentral scotomas, mild vision loss, and perifoveal reddish, wedge-shaped petalloid lesions on exam. Other risk factors commonly reported with AMN include viral prodrome, exposure to vasoconstrictive epinephrine or ephedrine, caffeine intake, and hypotensive shock. , To date, there has been one other report in the literature describing AMN in the setting of acute lymphoblastic leukemia (ALL). We herein report the first described case of AMN associated with acute promyelocytic leukemia (APL) with multimodal imaging.

Case presentation

A 37 year-old Asian-Indian male was admitted for fatigue and fever in the setting of viral pneumonia after routine labs detected pancytopenia (hemoglobin 5.4 g/dL, white cell count 3.0 K/UL, platelet 9.0 K/UL). His hematologic work-up was consistent with APL and all-trans retinoic acid therapy (ATRA) was initiated. His hospital course was complicated by disseminated intravascular coagulation, multifocal pneumonia, and acute respiratory distress requiring intubation. His inpatient treatment course consisted of two doses of idarubicin, arsenic trioxide, and ATRA therapy. Ophthalmology was initially consulted after globe flattening was detected on neuro-imaging. Examination at that time demonstrated significant papilledema indicative of intracranial hypertension secondary to ATRA therapy.

Following extubation, the patient complained of paracentral scotomas in the right eye. Fundus examination demonstrated Roth spots and peripapillary intra-retinal hemorrhages surrounding the nerve in both eyes. After management and stabilization of the patient’s complicated hospital course, the patient was discharged. He returned for follow up six weeks after initial onset of symptoms for further work-up and assessment of persistent paracentral scotomas of the right eye. Visual acuity was 20/20 in both eyes. Examination of the right fundus revealed intra-retinal hemorrhage within the superonasal macula and superior to the disc along with pigment mottling within the macula. His left eye revealed intra-retinal hemorrhage nasal to the disc and pigment mottling within the macula ( Fig. 1 A and B). The near infrared reflectance (NIR) clearly demonstrated well demarcated, wedge-shaped lesions pointing towards the fovea corresponding to the patient’s scotomas in the right eye and a single wedge shaped lesion in the left eye ( Fig. 2 A and C). Spectral domain optical coherence tomography (SD-OCT) imaging (Spectralis HRA + OCT, Heidelberg Engineering) showed thinning of the outer nuclear layer (ONL) and disruption of the ellipsoid zone (EZ) corresponding to the lesions seen on NIR ( Fig. 2 B and D). OCT angiography (OCT-A) using RTVue- XR Avanti system (Optovue Inc., Fremont, California) revealed loss of the deep capillary plexus (DCP) on 3 × 3 mm scans with attenuation of signal on both the enface and cross-sectional images in the regions corresponding to the NIR defects in the right and left eye ( Fig. 3 ). Fluorescein and indocyanine green angiography were unremarkable. Humphrey Visual Field 24–2 of the right eye demonstrated central and paracentral depression. Collectively these findings were consistent with a diagnosis of AMN.

Fig. 1

(A, B). Color fundus photographs of right and left eyes reveal intra-retinal hemorrhages and pigment mottling within macula.

Fig. 2

(A, C) Near infrared reflectance (NIR) of the right and left eye showing perifoveal wedge-shaped lesions (B, D) Spectral domain optical coherence tomography (SD-OCT) imaging reveals thinning of the outer nuclear layer (ONL) and disruption of the ellipsoid zone (EZ) corresponding to the lesions on NIR.

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Jul 10, 2021 | Posted by in OPHTHALMOLOGY | Comments Off on Acute macular neuroretinopathy associated with acute promyelocytic leukemia

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