Retinal Manifestations of Leukemia - Leukemic Retinopathy
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Leukemic Retinopathy is the most common ocular manifesation of Leukemia.
• Leukemia is a myeloproliferative disorder caused by an abnormal proliferation of immature leucocytes. Intraocular involvement in leukemia can be upto 90%.
• Both acute and chronic leukemia can cause ocular manifestations.
• Retina is the most common ocular tissue to be involved in leukemia.
• Leukemic retinopathy was first described by Liebriech in 1861 
• Retinal manifestations are usually due to indirect complications of leukemia especially due to hematological abnormalities (Figure 1)
Ocular complications of leukemia can either be :
1. Primary or Direct due to infiltration by leukemic tumour cells
2. Indirect/ Secondary
- Hematological abnormalities: Anemia, Thrombocytopenia
- CNS involvement
- Opportunistic infections
- Chemotherapy related
Prolonged leukocytosis increased number of circulating platelets --> Increased whole blood viscosity --> Reduced blood flow and vascular stagnation --> Peripheral capillary dropout and microaneurysm formation --> Proliferative retinopathy
• History of leukemia or any other malignancies- acute/chronic
• History of any other systemic illnesses
• History of treatment such as chemotherapy
• Visual loss - Onset, Duration
• Seeing a red shower of dots/ black shadow
• Visual field defects
1. Dilated and tortuous veins – early manifestations due to hematological disturbances 
2. Retinal hemorrhages at all levels – especially in the posterior pole, and extension to the vitreous
• Preretinal hemorrhages
• Superficial hemorrhages
• Dot and blot hemorrhages
• White centered hemorrhages/ Roth Spots (Figure 2)
• Sub-ILM hemorrhages
• Subhyaloid hemorrhage (Figure 3)
• Vitreous hemorrhage
ROTH SPOTS  (Figure 2)
White centered hemorrhages known as ‘Roth spots’ result from retinal capillary rupture and extrusion of whole blood. This is followed by platelet adhesion to the damaged endothelium initiating a coagulation cascade and formation of a white lesion in the centre of the hemorrhage which is a platelet-fibrin thrombus. In leukemia, the white centre could correspond to an accumulation of leukemic cells.
3. Cotton wool spots (Figure 4)
Secondary to clusters of cells occluding retinal arterioles, not usually associated with hematological parameters
4. Leukemic Infiltrates:
• It is generally associated with high blood counts, fulminant disease and early mortality.
• Usually, but not always seen with surrounding hemorrhage 
• Grey-white streaks along vessels caused by local perivascular infiltrates.
• Large grey, white nodules of varying sizes in the retina.
• Subretinal infiltrates- referred to as subretinal hypopyon
• Optic nerve infiltration
• Choroidal infiltrates – Serous retinal detachments, RPE detachments, discrete choroidal masses
• Vitreous infiltrates: Internal limiting membrane acts as an effective barrier for leukemic cells infiltration. But they can rarely invade the vitreous from the optic disc. Can be associated with vitreous hemorrhage. 
5. Vascular occlusions- Bilateral CRVO due to hyperviscocity (Figure 5)
6. Peripheral Microaneurysms- due to viscosity of elevated WBC count, does not correlate with hemoglobin or platelet level
7. Peripheral neovascularization similar to sickle cell anemia seen in CML- associated with chronic elevated WBC count
8. Retinal detachments
9. Vitreous infiltrates
10. Choroidal infiltrates
11. Opportunistic infections – CMV retinitis, Ocular toxoplasmosis, Viral retinitis
12. Optic nerve involvement
- History of leukemia
- Detailed fundus evaluation
- Laboratory tests
- Complete blood count – to look for anemia, thrombocytopenia, pancytopenia
- Peripheral blood smear- for blast cells
- Bone marrow biopsy – infiltration by blast cells
- Flow cytometry and immunophenotyping
Treatment of underlying leukemia by chemotherapy, immunotherapy or radiotherapy.
The 5-year survival rate was found to be significantly lower in those with leukemic retinopathy on presentation than in those without ophthalmic involvement (21.4% vs. 45.7%).
Patients with clinical leukemic retinopathy may have more aggressive systemic disease that might lead to a worse prognosis. 
- Liebreich: Atlas, 1863, as quoted inW.S. Duke-Elder Text-book of Ophthalmology vol. III, Mosby, St Louis (1941), Article 2740
- Sharma T, Grewal J, Gupta S, Murray PI. Ophthalmic manifestations of acute leukaemias: the ophthalmologist's role. Eye. 2004 Jul;18(7):663-72.
- Rosenthal AR. Ocular manifestations of leukemia: a review. Ophthalmology. 1983 Aug 1;90(8):899-905.
- Ballantyne AJ, Michaelson IC. Textbook of the Fundus of the Eye. Williams and Wilkins: Baltimore, 1970; 290–292.
- Allen RA, Straatsma BR. Ocular involvement in leukaemia and allied disorders. Arch Ophthalmol 1961; 66: 490–508.
- Kuwabara T, Aiello L. Leukaemic military nodule in retina. Arch Ophthalmol 1964; 72: 494–497.
- Retina E-Book: 3 Volume Set By Charles P. Wilkinson, David R. Hinton, SriniVas R. Sadda, Peter Wiedemann, Stephen J. Ryan