Atypical Proliferative Retinopathy as the Presenting Feature of Chronic Myeloid Leukemia in a Patient With Diabetes Mellitus

Abstract

Purpose: To describe a case of proliferative retinopathy as the presenting manifestation of chronic myeloid leukemia in a patient with poorly controlled diabetes mellitus (DM). Undiagnosed chronic myeloid leukemia in a patient with pre-existing poorly controlled DM is rarely encountered but must be recognized to treat appropriately with systemic chemotherapy. Significant fundus finding overlaps with DM making the recognition of chronic myeloid leukemia challenging.Methods: Case report.Results: Fundoscopy revealed scattered dot-blot hemorrhages, venous beading, and numerous Roth spots in all quadrants, in both eyes. In the right eye, there was also a vitreous hemorrhage with evidence of neovascularization near the inferior arcade. Intravenous fluorescein angiography showed significant peripheral capillary nonperfusion without evidence of exudation in both eyes. No macular edema was observed on optical coherence tomography. A review of systems and physical examination was negative for constitutional symptoms, lymphadenopathy, organomegaly, and other symptoms. Retinal findings prompted a complete blood count, which revealed significant leukocytosis. A bone marrow biopsy confirmed a diagnosis of chronic myeloid leukemia. Systemic chemotherapy and pan-retinal photocoagulation successfully normalized the leukocyte count and resolved the vitreous hemorrhage and neovascularization. Conclusion:The presence of numerous Roth spots in all quadrants, extensive areas of capillary nonperfusion on intravenous fluorescein angiography, and neovascularization in the absence of exudation or macular edema should prompt investigations to rule out hematologic disorders.

Bilateral vision loss as initial presentation of chronic myeloid leukemia in a young adult: A case report and review of the literature

“…Roth spots were once believed to be pathognomonic for acute bacterial endocarditis but have since been reported in anemia, leukemia, and high altitude retinopathy. 17 , 23 , 24 To our knowledge, the presence of Roth spots in patients with visual disturbances as the only presenting symptom has only been reported in CML 6 , 8 , 10 , 13 , 14 ; however, several types of retinal hemorrhages can present in leukemic retinopathy, which may mimic other pathologies. 24 , 25…”

“…To date, this is one of only a few reported cases of bilateral blurry vision as the primary presenting symptom of CML in a young adult. 6 , 7 , 8 , 9 , 10 , 11 , 12 , 13 , 14 , 15 , 16…”

“… 5 Of the eleven mentioned cases, vitreous hemorrhage, preretinal hemorrhage, optic disc hemorrhage, macular hemorrhage, dot and blot hemorrhage, flame-shaped hemorrhage, Roth spots, intraretinal hemorrhage and subhyaloid hemorrhage have all been documented. 6 , 7 , 8 , 9 , 10 , 11 , 12 , 13 , 14 , 15 , 16 Other reported initial ocular abnormalities include tortuous and dilated veins, neovascularization of the retina, optic disc edema, retinal detachment, and leukemic infiltrates, which were less common than retinal hemorrhage. 6 , 7 , 8 , 9 , 10 , 11 , 12 , 13 , 14 , 15 , 16 Kincaid and Green 5 suggested that microaneurysms and retinal neovascularization, comparable to what is seen in sickle cell anemia, could be specific to the chronic leukemia subtypes.…”

“…6 , 7 , 8 , 9 , 10 , 11 , 12 , 13 , 14 , 15 , 16 Other reported initial ocular abnormalities include tortuous and dilated veins, neovascularization of the retina, optic disc edema, retinal detachment, and leukemic infiltrates, which were less common than retinal hemorrhage. 6 , 7 , 8 , 9 , 10 , 11 , 12 , 13 , 14 , 15 , 16 Kincaid and Green 5 suggested that microaneurysms and retinal neovascularization, comparable to what is seen in sickle cell anemia, could be specific to the chronic leukemia subtypes. Our patient presented with vitreous hemorrhage, sub-inner limiting membrane hemorrhage, dilated tortuous vessels, macular hemorrhage, Roth spots, peripheral neovascularization, and intraretinal and pre-retinal hemorrhages.…”

“… 5 Patients with these changes have been well documented for decades; however, few cases have reported bilateral blurry vision as the primary presenting symptom of CML in a young adult. 5 , 6 , 7 , 8 , 9 , 10 , 11 , 12 , 13 , 14 , 15 , 16 We present a rare case of a young adult patient with recently diagnosed CML whose primary presenting symptom was bilateral blurry vision. We also include a review of other reported cases of CML with ocular findings as the initial presentation.…”