Chronic Lymphocytic Leukemia

Description
Leukemia is a systemic disease that can involve the eyes. It occurs in both children and adults. In that the eyes can be a relative sanctuary (a place to hide) for leukemic cells, intraocular recurrences have been reported after both total body irradiation and systemic chemotherapy. It is reasonable for all children with leukemia to have periodic eye examinations. Adults can also have leukemia and develop infiltration of the eyes. The case presented in this section demonstrates one such case.

Symptoms
Depending on the location of the leukemic infiltrates, patients will have different symptoms. Most patients are found to have retinal tumors with hemorrhage, optic nerve swelling (papilledema) or cells in the anterior chamber of the eye (hypopyon/hyphema). Leukemic tumor called "chloroma" can also form around the eyes. Leukemic Infiltrate of the Anterior Chamber - Hypopyon with Hyphema In this case, posterior uveal infiltration caused thickening of the choroid that moved the retina forward. This made the patient more far-sighted (hyperopic shift) due to a shortening of the distance between the cornea/lens complex and the retina. The front of the eye was also infiltrated by leukemic cells. Thickening of the iris, ciliary body and anterior uvea induced a secondary "narrow angle" glaucoma. The symptoms of glaucoma include seeing haloes around lights, headache (brow-ache) and eye pain.

A diffusely thickened iris secondary to leukemia.

Angle closure caused bilateral secondary glaucoma. High frequency anterior ultrasound reveals anterior uveal thickening consistent with infiltration. Anterior rotation of the iris and ciliary body causing apposition of the iris with the corneal-angle closure as documented with ultrasonography. B-scan ultrasound reveals posterior choroidal thickening consistent with infiltrative disease. The chorioretinal thickness is measured to be 2.4 mm.

Diagnosis
The clinical diagnosis of infiltrative ocular leukemia is adequate for patients with typical findings and a history of systemic leukemia. When proof of recurrence is needed to proceed with systemic or local therapy, a biopsy may be performed for a cytologic or histopathologic diagnosis.

Treatments
The treatment of intraocular leukemia is (in part) dependent on the treatment of the patients systemic disease. For example, in the clinical situation where the eye findings are not posing an immediate threat to vision, your physician my wait for systemic therapies to resolve the ocular disease. In cases where all standard treatments have been unsuccessful, patients will be treated with local radiation therapy. Our older patient was emergently treated with 24 Gy external beam radiation therapy. This decision was made to treat his tumor-associated narrow angle glaucoma. Since the effects of radiation therapy were not immediate, local measures (iridoplasty and medication) were employed to control his glaucoma. Months after irradiation, the medications were discontinued.