A unique case of chronic myeloid leukemia presenting as monocular vision loss with unilateral retinopathy

Abstract

PurposeTo report a case of unilateral leukemic retinopathy secondary to chronic myeloid leukemia (CML).ObservationsPatient presented to clinic with a visual acuity (VA) of 20/200 in the right eye (OD) after several months of progressive monocular vision loss and was found to have dense pre-retinal hemorrhage. Patient underwent 23-gauge pars plana vitrectomy to clear the preretinal hemorrhage along with a complex macula-off retinal detachment repair to address retinal tear and multilayer retinal hemorrhage. The patient was subsequently diagnosed with CML as she was found to be positive for the fusion protein of break point cluster gene (BCR) with Abelson tyrosine kinase (ABL1), BCR-ABL1, upon systemic work-up. Imatinib therapy resulted in complete hematologic and cytogenetic resolution after one month, however, the patient's vision remained unchanged six months after surgery.Conclusion and importanceTo the authors' knowledge, this is the first reported case of unilateral leukemic retinopathy secondary to low risk CML, as determined by the Sokal and Hasford prognostic scoring systems. CML should be included in the differential diagnosis of patients with progressive monocular vision loss with suspicious multi-layer retinal compromise.

Ophthalmologic manifestations as the initial presentation of chronic myeloid leukemia: A review

“…The median age was 37.05 years (range 27.5-49), and 67.5 % of patients were male. Most of the cases were initial presentations of CML (33 cases), and only five of them were in relapses 4,[8][9][10][11][12][13][14][15][16][17][18][19][20][21][22][23][25][26][27][28][29][30][31][32][35][36][37][39][40][41][42][43][44][45][46][47] . The retina was most often involved, with retinal hemorrhages as the most common finding.…”

“…Nine presented with unilateral CML-related ophthalmic findings. 12,17,20,22,27,32,33,39,42 Eight of these had unilaterallydecreased of vision, and 1 had normal vision. 12 Thirty-one patients presented with bilateral ophthalmic findings 4,[8][9][10][11][13][14][15][16]18,19,[23][24][25][26][28][29][30][31][32][34][35][36][37][38][40][41][42]44,46 .…”

“…In the posterior segment vasculopathy secondary to CML is more common than direct leukemic infiltration (Table 1). The most common findings are leukemic retinopathy (Roth spots, cotton wool spots, leukemic infiltrates, preretinal hemorrhage, intraretinal hemorrhage, subretinal hemorrhage, dilated tortuous vessels, optic disc hyperemia, and retinal vascular sheathing 9,10,15,20,22,[24][25][26]28,31,32,34,[37][38][39][40]42,44,46 .…”

“…Fundus fluorescein angiography can be helpful in revealing occult neovascularization, ischemia, RPE pathology 4,8,12,14,15,23,24,29,33,35,36,44 . B scan ultrasonography can help diagnose retinal detachment, subretinal/ choroidal pathologies such as mass and hemorrhage 11,39 . Visual evoked potentials (VEP) may help identify optic nerve involvement 12 .…”

“…Panretinal photocoagulation was done for proliferative leukemic retinopathy 4,8,14,29,35,36 . Other cases with non-resolving vitreous hemorrhage, massive subretinal hemorrhage, and tractional retinal detachment required pars plana vitrectomy 4,8,11,18,23,29,39 . One case of bilateral central retinal vein occlusion with macular edema required intravitreal bevacizumab injection 35 .…”