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The 2 main types of ocular involvement include direct leukemic infiltration of ocular tissues and secondary leukemic retinopathy (e.g., retinal hemorrhages, cotton wool spots) from hematological complications of leukemia like anemia and hyperviscosity.
However, CML rarely displays posterior pole proliferative retinopathy or optic disc neovascularization. We report a case of CML that presented with bilateral optic disc neovascularization.
A healthy 46-year-old white male presented with decreased vision (20/30 OD, 20/25 OS) associated with headaches. Dilated fundus examination revealed bilateral optic disc neovascularization, white-centered retinal hemorrhages, venous dilation with areas of telangiectasis, and retinal neovascularization (Fig. 1). Fundus fluorescein angiography indicated leakage from optic nerve and retinal neovascularization with widespread areas of peripheral nonperfusion and some posterior capillary dropout in both eyes (Fig. 1). The patient had an elevated white blood cell count of 243 900 cells/mL (normal 4300–5700 cells/mL) with a hemoglobin of 7.9 g/dL (normal 13.8–17.2 g/dL). Magnetic resonance imaging of the brain revealed a frontotemporal lobe lesion that was excised by craniotomy and determined to be a granulocytic sarcoma of myeloid cells. A BCR-ABL translocation [t(9:22)(q34;q11.2)] was present on fluorescence in situ hybridization resulting in the formation of the Philadelphia chromosome. The diagnosis of CML with severe leukemic retinopathy was made and the patient was treated with the tyrosine kinase inhibitor dasatinib.
Over the course of 5 months, despite good control of systemic disease, his CML retinopathy progressed with recurrent preretinal and vitreous hemorrhages in both eyes (Fig. 2). Treatment was initially performed with bilateral full panretinal photocoagulation. Despite photocoagulation, 3 months later, our patient developed bilateral tractional retinal detachments (Fig. 2). Pars plana vitrectomy including membranectomy, endophotocoagulation, and air-fluid exchange followed by gas-air exchange with 16% perfluoropropane (C3F8) was performed in both eyes. The final vision was 20/25 OU with no signs of active retinopathy (Fig. 2). The patient continues to be in remission of his CML.
Peripheral neovascularization is common in patients with CML and was first described in 1968.
Hyperviscosity causes peripheral nonperfusion and ischemia resulting in capillary dropout with possible formation of neovascularization. This predilection for the retinal periphery in CML can present with advanced proliferative retinopathy
In contrast with proliferative diabetic retinopathy, it is extensively reported that the neovascularization observed in patients with CML typically remains in the midperiphery to far periphery and spares the optic nerve.
Our case illustrates that, although rare, CML retinopathy may initially present with bilateral optic disc neovascularization. The case is instructive that once the capillary dropout occurred, the neovascularization and traction developed despite good control of the systemic disease with normalization of the high white cell count. The rapid progression of retinopathy also highlights the importance of prompt diagnosis and treatment, and we recommend peripheral blood analysis be ordered in patients who present with white-centered hemorrhages or retinal hemorrhages not in keeping with known ocular or systemic diseases. As a corollary, we suggest to our medicine colleagues that all patients with newly diagnosed leukemia of any type be referred for ocular examination regardless of whether they are symptomatic.
D.R.P.A. has received honoraria from Allergan, Alcon, Genentech, and Novartis.
Supported by: This work was supported by the Retina Foundation of Canada (D.R.P.A.).