Hematolymphoid malignancies with intraocular intravascular involvement: report of 2 cases

The interaction between the endothelium and malignant hematolymphoid cells within vessels of the eye can result in focal or diffuse intravascular pathology. As a result, correlation of these findings with specific clinical and ophthalmologic features can vary. We review the ophthalmic findings in two cases of hematolymphoid malignancies limited to the intravascular space and review published literature on this topic. In cases of intravascular large B-cell lymphoma, underexpression of β1-integrin and intercellular adhesion molecule-1 by the cells of intravascular large B-cell lymphoma results in diffuse ocular vascular involvement. The widespread degree of intravascular involvement correlates with clinical ophthalmologic findings and may lead to retinal and choroidal detachment that is observed postmortem. Conversely, in the context of acute leukemia, induced overexpression of certain adhesion molecules (intercellular adhesion molecule-1 and vascular cell adhesion molecule-1) in the endothelium of certain vascular beds may result in leukostasis with only selective (choroidal) ocular vessel involvement. As a result of only focal vascular activation and adhesion in the orbit, the gross findings in these cases are minimal and may not correlate with clinical ophthalmologic findings.