Hematolymphoid malignancies with intraocular intravascular involvement: report of 2 cases

Ann Diagn Pathol. 2011 Aug;15(4):286-90. doi: 10.1016/j.anndiagpath.2010.04.006. Epub 2010 Jul 8.


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.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Aged
  • Blood Vessels / metabolism
  • Blood Vessels / pathology*
  • Eye / blood supply
  • Eye / pathology*
  • Eye Neoplasms / blood supply
  • Eye Neoplasms / metabolism
  • Eye Neoplasms / pathology*
  • Humans
  • Intercellular Adhesion Molecule-1 / metabolism
  • Leukostasis / pathology
  • Lymphoma, Large B-Cell, Diffuse / metabolism
  • Lymphoma, Large B-Cell, Diffuse / pathology*
  • Male
  • Precursor Cell Lymphoblastic Leukemia-Lymphoma / metabolism
  • Precursor Cell Lymphoblastic Leukemia-Lymphoma / pathology*
  • Vascular Cell Adhesion Molecule-1 / metabolism
  • Young Adult


  • Vascular Cell Adhesion Molecule-1
  • Intercellular Adhesion Molecule-1