Table.
Summary of the clinical, morphological, immunophenotypical and genotypical features known to date for each of the various intraocular lymphoma subtypes
| Intraocular Lymphoma Type |
Retinal | Choroidal Primary: |
Choroidal Secondary: |
Iridal | Ciliary body |
|
|---|---|---|---|---|---|---|
| Clinical Features | 60–70 years “Floaters” Painless decrease in VA Subretinal infiltrates Often bilateral RPE changes on FA CNS involvement (70–80% of pts) |
50–60 years Blurring of vision Metamorphopsia Clear vitreous Diffuse thickening of choroids Usually unilateral Extraocular extension frequent No CNS involvement |
Previous history of systemic NHL Decrease in VA Possibly bilateral |
Pain Redness Photophobia Pseudohypopyon Usually unilateral Often ultimate systemic dissemination |
Raised IOP Ciliary body mass |
|
| Most Common Subtype(WHO) Immunoprofile |
DLBCL CD79a+ CD20+ PAX5+ BCL2+ BCL6+/− MUM1/IRF4+ CD10−/+ Ki-67 rate: high (>80%) |
EMZL CD79a+ CD20+ BCL2+ CD43 +/− IgM + CD5- CD23- CyclinD1- Low Ki-67 rate: 5–15% |
Dependent on systemic NHL Dependant on systemic NHL |
DLBCL CD20+ Ki-67 rate: high (>80%) |
TCL NOS CD3+ CD4+ Ki-67 rate: moderate (40–60%) |
EMZL CD79a+ CD20 + BCL-2 + CD43 +/− IgM + CD5- CD23- CyclinD1- Low Ki-67 rate: 5–15% |
| Genotype | High somatic IgH mutation load Few ongoing somatic mutations Chromosomal translocations: t(14;18)(q31;q21) |
Moderate somatic IgH mutation load Few ongoing somatic mutations Chromosomal abnormalities: t(11;18)(q21;q21) |
Dependant on systemic NHL |
Not known | Not known | Not known |
| Putative Cell of Origin |
2 different types?: a) Early post-germinal centre B cell = DLBCL of ABC type? b) Germinal centre cell = DLBCL of GCB type ? |
Post-germinal centre (memory) B cell |
Dependant on systemic NHL |
Not known | Peripheral T-cell |
Post-germinal centre (memory) B cell |
Key: DLBCL=diffuse large B-cell lymphoma; EMZL=extranodal marginal zone B-cell lymphoma;NHL=NonHodgkin’s lymphoma;CD=cluster of differentiation; ABC = activated B-cell type; GCB = germinal centre B-cell type; t(N1;N2) = chromosomal translocation between chromosome N1 and N2. WHO = WHO lymphoma classification system.