Leukemia Retinopathy

Leukemia Retinopathy
SYMPTOMS Non-proliferative: Typically asymptomatic but may report Central vision loss and Metamorphopsia in the presence of cystoid macular edema, Central vision loss and Peripheral vision loss in the presence of retinal vein occlusions, infiltration of the the retina/choroid/optic nerve/vitreous, and opportunistic infections such as toxoplasmosis
Proliferative: Central vision loss and Metamorphopsia in the presence of cystoid macular edema, Central vision loss and Peripheral vision loss in the presence of retinal vein occlusions, infiltration of the the retina/choroid/optic nerve/vitreous, subhyaloid/preretinal/intravitreal hemorrhages, and opportunistic infections such as toxoplasmosis
SIGNS Typically bilateral
Non-proliferative signs: Dilated and tortuous veins/venules, Microaneurysms, Dot/blot hemorrhages, Flame hemorrhages, Roth spots, Cotton wool spots, Cystoid macular edema, Retinal vein occlusions, Infiltration of the retina/choroid/optic nerve/vitreous, Signs of opportunistic infections such as toxoplasmosis
Proliferative signs: All of the signs seen with non-proliferative leukemia retinopathy and NVD, NVE, Subhyaloid hemorrhages, Preretinal hemorrhages, Intravitreal hemorrhages
WORK-UP Pupils, EOMs, Full eye exam with dilated retinal exam, OCT analysis of the macula (signs of cystoid macular edema are best seen with an OCT), OCT analysis of the optic nerve, OCT-Angiography, Fluorescein Angiography, Fundus photos, B-scan ultrasound (if unable to view the retina), Watzke-Allen test, Macular photostress test, Amsler grid
TREATMENT Give take home Amsler grid in order to monitor for change
Patient needs to follow up with their primary care provider in order to diagnose, evaluate, and treat the leukemia
Patient needs to use caution with any strenuous exercise or activities in the presence of proliferative leukemia retinopathy
Patient should sleep with their head elevated in presence of a preretinal, subhyaloid, and/or intravitreal hemorrhage
Consider discontinuing or lowering the dosage of any blood thinners in the presence of proliferative leukemia retinopathy (needs to be discussed with PCP)
Non-proliferative leukemia retinopathy: Needs to be monitored closely. There is no ocular treatment
Non-proliferative leukemia retinopathy with cystoid macular edema: Refer to retinal specialist ASAP for treatment
Proliferative leukemia retinopathy with or without cystoid macular edema: Refer to retinal specialist ASAP for treatment
Treatment for proliferative leukemia retinopathy includes intravitreal anti-VEGF injections, pan retinal photocoagulation, and a vitrectomy
Patients with opportunistic infections involving the back of the eye need to be referred to a retinal specialist ASAP for treatment as well
FOLLOW-UP If there is no evidence of retinopathy, patient should be seen back in 6-12 months
If there is evidence of non-proliferative leukemia retinopathy, patient should be seen back in 1-2 months
If macula is stable (in a patient with non-proliferative leukemia retinopathy) following treatment by retinal specialist, patient should be seen back in 3-4 months
Once proliferative leukemia retinopathy becomes involutional or quiescent and the retina and macula is stable following treatment by the retinal specialist, patient should be seen back every 4-6 months
ADDITIONAL LAB | TESTS The patient needs to follow-up with their PCP for additional testing if not already done which includes the following: CBC with differential, Peripheral blood smear
ETIOLOGY Leukemia causes accumulation of abnormal white blood cells in circulation (leukostasis), anemia, and thrombocytopenia. This causes hyperviscosity of the blood as well as a decrease of oxygen in the blood stream. Eventually there will be damage to the retinal vasculature which will cause leakage as well as a decrease in blood flow, oxygen, and perfusion
DIFFERENTIAL DX Diabetic retinopathy, Hypertensive retinopathy, Anemia retinopathy, HIV retinopathy, Sickle cell retinopathy
NOTES Ocular signs and symptoms may be the initial presentation of leukemia is 3.6% of patients
The most common area of the eye affected by leukemia is the retina
Dot/blot hemorrhages, flame hemorrhages, and roth spots are the most common sign seen in patients with leukemia retinopathy
Leukemia retinopathy is associated with more aggressive systemic disease
Myeloid leukemia is the most common type of leukemia associated with ocular findings
Leukemia Retinopathy: Fundus photo demonstrating non-proliferative leukemia retinopathy with multiple roth spots https://imagebank.asrs.org/file/1164/leukemic-retinopathy