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Choroidal lymphoma

PRIMARY CHOROIDAL LYMPHOMA

Primary choroidal lymphoma is rare. Previously it was termed 'uveal pseudotumour' or 'reactive lymphoid hyperplasia'. In most patients, this is a low-grade (i.e., non-aggressive) lymphoma arising in the choroid of one eye. It is commoner in men than women.

 

It usually consists of B-cells of “extranodal marginal zone B-cell subtype, apparently arising from a marginal zone (memory) B-cell.

 

The condition causes a pink swelling, often with retinal detachment. The tumour tends to grow out of the eye, near the optic nerve and under the conjunctiva. The vitreous remains clear.

 

 

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Fundus appearance with diffuse, pink swelling and obscuration of normal choroidal vessels.

 

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Ultrasound B-scan showing diffuse choroidal lymphoma with low internal acoustic reflectivity and extraocular nodules adjacent to the optic nerve

 

Histology shows small atypical lymphocytes, consisting of centrocyte-like cells, monocytoid cells, plasmacytoid cells, some with obvious Dutcher and Russell bodies, as well as occasional blasts. The neoplastic lymphocytes express B-cell antigens (e.g., CD79a and CD20), BCL-2 and, in some cases, demonstrate an aberrant expression of the T-cell antigen CD43. A monotypical expression for Ig-kappa and IgM is usually seen. The tumour cell Ki-67 growth fraction is typically small (ca. 10-15%).

 

Treatment

Most patients do well with a small course of radiotherapy.

 

Prognosis

The prognosis for survival is good.

 

SECONDARY CHOROIDAL LYMPHOMA

This is the most common manifestation of secondary intraocular lymphoma, with predominant retinal involvement and anterior segment disease being rare.

 

The most common lymphoma sub-type is diffuse large B-cell lymphoma.

 

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Secondary uveal lymphoma affecting both eyes

 

Treatment

Treatment is determined by the underlying systemic condition.

 

FURTHER READING

Coupland SE, Damato B. Understanding intraocular lymphomas. Clin Experiment Ophthalmol. 2008 Aug;36(6):564-78.