Retinopathy in blood diseases

Retinopathy with anemia

Anemias are a group of blood disorders characterized by a decrease in the volume of circulating red blood cells and/or hemoglobin. Retinal changes in anemias usually occur without consequences and are rarely of diagnostic value.

Retinopathy is characterized by hemorrhages, sometimes with a white spot in the center (Roth spots, cotton wool spots and tortuosity of branches).

The duration and type of anemia do not affect the appearance of these changes, which are more characteristic of concomitant thrombocytopenia.

Flame-like hemorrhages and cotton-wool lesions may appear in the absence of other blood abnormalities.

The tortuosity of the veins depends on the severity of the anemia. Roth spots are fibrinous thrombi that clog vascular ruptures. They can be found in bacterial endocarditis and leukemia.

Optic neuropathy with central scotomas may occur in pernicious anemia. If the patient is not treated with vitamin B12, persistent optic atrophy develops. Pernicious anemia may cause dementia, peripheral neuropathy, and subacute combined degeneration of the spinal cord involving the posterior and lateral funiculi.

Retinopathy in leukemia

Leukemias are classified as a group of neoplastic changes characterized by proliferation of white blood cells. Eye damage is more often observed in the acute form, less often in the chronic form, with possible involvement of different structures of the visual organ. However, it is important to distinguish the rather rare phenomena of infiltration in primary leukemias from the more frequent secondary changes associated with anemia, thrombocytopenia, increased viscosity and opportunistic infections.

Retinopathy is relatively common. Changes are similar to anemia with flame-like hemorrhages, Roth spots, cotton-wool spots. The latter may be due to leukemic infiltration, secondary anemia, or increased viscosity. Peripheral retinal pevascularization is a common manifestation of chronic myeloid leukemia. Less commonly, secondary choroidal infiltration may result in leukemic pigment epitheliopathy, known as leopard spots on the fundus.

Other eye manifestations

• Orbital involvement, more common in children.
• Thinning of the iris, iritis and pseudohypopyon.
• Spontaneous subconjunctival hemorrhage or hyphema.
• Optic neuropathy due to infiltration of the optic nerve.

Conditions of increased blood viscosity

Hyperviscosity states are a group of various rare disorders characterized by increased blood viscosity due to polycythemia or abnormal plasma proteins, as in Waldenstrom's macroglobulinemia and myeloma. Retinopathy is characterized by venous dilation, segmentation and tortuosity, and retinal hemorrhages.