What is Primary Intraocular Lymphoma?
Primary intraocular lymphoma (PIOL) is a rare form of cancer that originates inside the eye, most often in the vitreous humour (the clear, gel-like substance in the center of the eye) or the retina (the light-sensitive tissue at the back of the eye). PIOL is a type of non-Hodgkin lymphoma, typically involving B cells, which are part of the body’s immune system.
PIOL can be difficult to diagnose because its symptoms often mimic those of more common eye conditions such as infections or inflammation. However, unlike those conditions, PIOL is a serious cancer that requires specialised care. If left untreated, it can spread to the brain or spinal cord, making early diagnosis and treatment critical.
Although still considered rare, the number of PIOL cases has been increasing due to improved awareness and diagnostic capabilities. Among cancers that develop exclusively in the eye, PIOL is the most common type of lymphoma.
Who is at Risk?
The exact cause of PIOL remains unknown. However, certain risk factors may increase the likelihood of developing the disease:
- Older age, typically between 60 and 85 years
- Immunosuppression, such as in individuals living with HIV/AIDS or those who have received organ transplants
Signs and Symptoms
People with PIOL may experience symptoms such as:
- Blurred or decreased vision
- Floaters (seeing spots, shadows, or cobweb-like shapes)
- Eye pain
- Redness in the eye
- Sensitivity to light
These symptoms can be mistaken for uveitis or other inflammatory eye diseases, which can delay diagnosis.
How is PIOL Diagnosed?
A combination of tests is usually needed to confirm the diagnosis, including:
- Comprehensive eye examination – To assess the appearance and health of the eye
- Fundoscopy and MRI – Imaging of the eyes and brain to check for abnormalities
- Vitreous biopsy – A sample of the vitreous humour is collected and examined for lymphoma cells
- Lumbar puncture (spinal tap) – To analyse cerebrospinal fluid and detect possible spread to the brain or spinal cord
- Genetic testing – To identify specific markers of lymphoma cells
Treatment Options
Treatment is based on the location and extent of the cancer, especially whether it has spread beyond the eye.
Common treatment strategies include:
- Intravitreal injections – Direct injections of anti-cancer medications such as methotrexate or rituximab into the eye
- Chemotherapy – Often used if the lymphoma has spread to the brain or is at high risk of spreading
- Radiation therapy – Targeted radiation may be used to destroy lymphoma cells in the eye or brain
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