Primary Vitreoretinal Lymphoma (PVRL)

Primary vitreoretinal lymphoma is a cancer of immune cells called lymphocytes that starts inside the eye, mainly in the vitreous gel (the clear jelly that fills the eye) and the retina (the light-sensing layer at the back of the eye). In most people, this cancer comes from B cells, which are a type of white blood cell that normally helps fight infection. The cancer cells float in the vitreous gel and collect under or inside the retina, where they can quietly grow. The disease often looks like inflammation inside the eye at first, so it can mimic uveitis (eye inflammation). This is why doctors sometimes call it a “masquerade syndrome,” because it pretends to be inflammation when it is actually cancer.

PVRL is closely linked to primary central nervous system lymphoma (PCNSL). Some people have the eye disease first and later develop lymphoma in the brain. Some have brain disease first and later develop the eye disease. Some have both together. This connection is important because it changes the tests, the treatment plan, and the follow-up schedule. PVRL most often affects older adults, and both eyes are commonly involved, but sometimes it begins in only one eye.

PVRL tends to respond to steroids at the beginning because steroids reduce the inflammation around the tumor. This temporary response can make the disease harder to recognize, because the eye gets better for a short time and then gets worse again. Over time, the cancer cells can blur vision, create floaters, and damage the retina, which can lead to vision loss if the disease is not found and treated.

The lymphoma cells shed into the vitreous and make the gel cloudy. They also gather under the retina and beneath the retinal pigment epithelium (RPE), which is a thin support layer under the retina. These small cancer clumps can block light, irritate the retina, and release chemicals that change the way the retina works. The cancer cells often make a lot of IL-10, which is a chemical that calms immune cells and helps the tumor hide. Because the eye is an immune-privileged place (the immune system is gentler there to protect vision), the cancer can hide for months by pretending to be regular inflammation.

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Types of Primary Vitreoretinal Lymphoma

Types help doctors describe what they see, how the cells look, and how the disease behaves. The words here are simple so each type is clear.

1. B-cell PVRL
   This is the most common type. The cancer comes from B cells. Under the microscope, it most often looks like diffuse large B-cell lymphoma. This type usually makes high IL-10 and shows certain B-cell markers on special stains.

2. T-cell PVRL (rare)
   This type comes from T cells instead of B cells. It is much less common. It may behave differently and may need a different test panel to confirm the diagnosis.

3. Vitreous-predominant PVRL
   The cancer cells are mainly floating in the vitreous gel. People often notice floaters and hazy vision. The retina can look fairly normal early on.

4. Sub-RPE/Retinal-predominant PVRL
   The cancer cells sit under or within the retina and the RPE layer. Doctors may see creamy or yellowish spots, fine lines, or tiny elevations under the retina on imaging tests.

5. Isolated ocular PVRL
   The disease is found only in the eyes at the start. People with this pattern still need brain imaging and close follow-up, because the brain can become involved later.

6. Concurrent ocular and CNS PVRL
   The eyes and the brain or spinal fluid are involved at the same time. This pattern requires combined eye and brain evaluation and a coordinated treatment plan.

7. Unilateral PVRL
   The disease starts in one eye, but doctors still check the other eye often, because PVRL can become bilateral over time.

8. Bilateral PVRL
   Both eyes are involved. This is common in PVRL and helps doctors suspect the diagnosis when both eyes show similar findings.

9. Steroid-responsive masquerade PVRL
   Symptoms and exam findings improve briefly with steroids and then return. This pattern can delay diagnosis, so doctors think about PVRL when inflammation keeps coming back.

10. Genetically characterized PVRL
    Some tumors show specific gene changes (for example, changes that keep B-cell signals turned on). These details help confirm the diagnosis and may guide targeted treatments.

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Causes

Important note: The exact cause of PVRL is not fully known. The items below are biological drivers, risk conditions, and associations that researchers have found. Not every person with PVRL has these factors, and having one does not mean you will get PVRL.

1. Getting older
   As we age, B cells go through many cycles of change, and random DNA errors can build up. These errors sometimes create cancer behavior.

2. Random DNA changes in B cells
   B cells edit their DNA as they learn to fight germs. Rarely, this process creates harmful mutations that can lead to lymphoma in the eye.

3. A key mutation in MYD88 (often called L265P)
   This mutation can turn on growth signals inside B cells and help them survive when they should not.

4. Changes in CD79B and the B-cell receptor pathway
   These changes boost B-cell signaling, which can push cells to grow and resist normal controls.

5. Constant “ON” NF-κB signaling
   NF-κB is a master switch for survival. When it stays on, cells can live too long and act like cancer.

6. Eye’s immune-privileged status
   The eye naturally softens immune attacks to protect vision. This gentle setting can also help lymphoma cells hide.

7. High IL-10 environment
   The tumor often makes IL-10, which calms immune cells. This helps the cancer evade detection.

8. Weak immune system from HIV/AIDS
   A weakened immune system can make it easier for abnormal B cells to grow without being stopped.

9. Weak immune system after organ transplant
   Strong anti-rejection medicines can lower immune surveillance, which raises lymphoma risk in general, including rare eye cases.

10. Long-term use of strong immunosuppressive drugs
    Some medicines used for autoimmune diseases quiet the immune system, which can let abnormal cells expand.

11. Long-standing autoimmune disease
    Ongoing immune activity and repeated treatment may stress B cells and increase the chance of harmful DNA changes.

12. Chronic antigen stimulation
    B cells that face long-lasting triggers may over-proliferate, making DNA mistakes more likely.

13. Inherited differences in immune control
    Some people may carry genetic backgrounds that slightly shift risk, though no single pattern explains most cases.

14. Defects in DNA repair
    If repair systems are less efficient, B cells can accumulate mutations that support lymphoma behavior.

15. Shared biology with primary CNS lymphoma
    The eye and brain are both immune-gentle sites. Lymphoma cells that grow in the brain can also live in the eye, and vice versa.

16. Abnormal homing signals (chemokines/adhesion molecules)
    Changes in cell “GPS” signals can guide malignant B cells to settle in the eye tissues.

17. Support from the retinal environment
    Nearby cells, like retinal pigment epithelium, may release survival signals that help tumor cells persist.

18. Rare EBV-driven lymphoma in immunocompromised people
    In severely weakened immune systems, certain viruses like EBV can drive some lymphomas, though this is not the usual PVRL scenario.

19. Iatrogenic lymphoproliferation in autoimmune disease
    In rare cases, long treatment of autoimmune disease can lead to lymphoid overgrowth, which sometimes involves the eye.

20. Unknown causes
    In many people, no clear trigger is found. The disease likely results from several small factors acting together over time.

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Symptoms

1. Blurred vision
   Vision becomes smudged or hazy because cancer cells and inflammation cloud the vitreous and disturb the retina.

2. Floaters
   People see small moving spots or threads. These come from cells and debris drifting in the vitreous gel.

3. Painless, gradual vision loss
   The disease often progresses without pain, making it hard to notice until vision is clearly worse.

4. Vision that gets better with steroids, then worse again
   Steroids quiet inflammation, so vision improves briefly. When steroids stop, the cancer activity returns.

5. Light sensitivity (photophobia)
   The irritated retina and inflamed tissues can make bright light uncomfortable.

6. Poor contrast and washed-out colors
   Fine detail and color look dull, because the retina is not processing light normally.

7. Distorted vision (metamorphopsia)
   Straight lines look wavy, because abnormal spots under or within the retina bend the light path.

8. Dark or missing spots in vision (scotomas)
   Areas of the retina do not work well, so parts of the view may look dim or blank.

9. Trouble seeing at night
   Low-light vision can become weak, because the retina’s light-capturing cells are affected.

10. Mild redness or a “quiet” white eye
    The eye may be slightly red or even look normal. This lack of obvious redness can mislead observers.

11. Occasional eye ache or pressure
    Some people feel a dull pressure. Many feel no pain at all.

12. Reduced color discrimination
    Colors may lose richness, because damaged retina cells cannot process color well.

13. Fluctuating day-to-day vision
    Vision can change from day to day, depending on cell clusters, cloudiness, and inflammation levels.

14. Headache or neurologic symptoms (if CNS is involved)
    If the brain is involved, people can have headaches, confusion, memory changes, or other nerve problems.

15. One eye first, then both
    The disease can start in one eye and later involve both, which is common in PVRL.

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Diagnostic Tests

(grouped as Physical Exam, Manual Tests, Lab/Pathological Tests, Electrodiagnostic Tests, and Imaging Tests; each explained in simple words)

A) Physical Exam

1. General medical and neurologic exam
   The doctor checks overall health, balance, memory, and nerve function. This looks for signs of brain involvement and other causes of inflammation or vision loss.

2. External eye exam
   The doctor looks at the eyes and eyelids in normal light. The eye can appear quiet even when lymphoma is present, which raises suspicion when symptoms are not matching the calm appearance.

3. Pupil reactions (checking for RAPD)
   The doctor shines light to see how pupils respond. An afferent pupillary defect can point to optic nerve or retinal dysfunction, which supports the need for deeper testing.

B) Manual (Clinical) Eye Tests

4. Visual acuity testing
   Reading the chart shows how sharp vision is. Persistent blur that doesn’t match the mild exam can hint at masquerade disease like PVRL.

5. Amsler grid
   A small grid checks for wavy lines or missing areas. Distortion suggests retinal involvement from sub-retinal lymphoma spots.

6. Confrontation visual fields
   The doctor maps side vision by simple bedside testing. Missing patches can suggest retinal or nerve pathway dysfunction.

7. Slit-lamp biomicroscopy
   A microscope with a bright light lets the doctor see cells floating in the vitreous, check the front of the eye, and assess inflammation. Fine clumps of cells and vitreous haze raise concern for PVRL.

8. Dilated indirect ophthalmoscopy
   With the pupils dilated, the doctor examines the retina and RPE. Creamy lesions, sub-RPE deposits, and patchy changes can be seen in PVRL.

C) Laboratory & Pathological Tests

9. Diagnostic vitreous biopsy (cytology)
   A small sample of the vitreous gel is taken with a tiny instrument. A pathologist looks at cells under a microscope. Large, abnormal lymphocytes with certain features support the diagnosis. This test is central to confirming PVRL.

10. Cell block and immunohistochemistry (IHC)
    The fluid is processed into a cell block so it can be stained for markers. B-cell PVRL often shows B-cell markers and light-chain restriction. IHC proves the cell type.

11. Flow cytometry
    This test profiles the surface proteins of many cells very quickly. A clonal B-cell population supports lymphoma. It helps confirm and classify the disease.

12. Cytokine measurement (IL-10 and IL-6)
    The vitreous sample is tested for IL-10 and IL-6. In PVRL, IL-10 is often high, and the IL-10/IL-6 ratio can be greater than one, which supports the diagnosis. This is not the only test, but it is helpful.

13. Molecular testing for lymphoma-related mutations
    Tests look for changes in genes that drive lymphoma growth, such as MYD88 and others. Finding these mutations in the eye sample adds strong evidence for PVRL.

14. Clonality testing (Ig heavy chain gene rearrangement PCR)
    This test checks if the B cells are all from one clone, which is a hallmark of lymphoma. A clonal pattern strongly supports PVRL.

D) Electrodiagnostic Tests

15. Electroretinography (ERG)
    ERG measures how the retina responds to light. Abnormal ERG shows that the retina’s function is impaired, which aligns with retinal lymphoma involvement.

16. Visual evoked potentials (VEP)
    VEP measures signals from the eye to the brain. Abnormalities can reflect retinal or optic pathway dysfunction, and may support the need for full CNS evaluation.

E) Imaging Tests

17. Optical coherence tomography (OCT)
    OCT makes thin cross-section pictures of the retina. Doctors can see sub-RPE deposits, hyper-reflective bands or nodules, and retinal layer changes that fit PVRL. OCT is painless and very informative.

18. Fluorescein angiography (FA)
    A small dye is injected into a vein in the arm, and retinal photos are taken as the dye circulates. FA can show leakage patterns and blockage areas caused by lymphoma under or within the retina.

19. Fundus autofluorescence and/or indocyanine green angiography (FAF/ICGA)
    FAF captures natural glow from retinal pigments and can reveal abnormal patches over lymphoma areas. ICGA uses a different dye to image deeper layers, helping show changes in the choroid and RPE.

20. MRI of brain and orbits
    MRI looks for lesions in the brain and checks the eye sockets. Because PVRL and brain lymphoma are often connected, MRI is critical to complete staging and planning.

Non-pharmacological treatments (therapies & other measures)

Below are supportive, practical steps that do not replace medical therapy but improve comfort, safety, and overall outcomes. Each item includes what it is, why it helps, and how it works.

1. Low-vision rehabilitation. A therapist teaches lighting, contrast, magnifiers, and device settings to make reading and mobility easier. Purpose: maintain independence. Mechanism: compensates for reduced retinal function by optimizing visual input to the brain.

2. Assistive technology training. Screen readers, large-print settings, text-to-speech, and high-contrast modes. Purpose: easier day-to-day tasks. Mechanism: enhances legibility and reduces eye strain.

3. Falls-prevention home adjustments. Better lighting, non-slip mats, marking stair edges. Purpose: reduce injury during periods of blurry or fluctuating vision. Mechanism: environmental hazard reduction.

4. Dry-eye care during treatment. Preservative-free tears and eyelid hygiene—especially with intravitreal therapy or after radiotherapy. Purpose: comfort, clearer vision. Mechanism: stabilizes the tear film and corneal surface.

5. Infection-risk reduction. Good hand/eye hygiene, avoiding contact lens wear when advised, prompt care for red/painful eyes. Purpose: prevent conjunctivitis or keratitis while on chemo/steroids. Mechanism: lowers pathogen exposure and supports local barriers.

6. Vaccination review (non-live when appropriate). Ask oncology team about influenza/COVID and other inactivated vaccines before/after systemic therapy. Purpose: reduce severe infections during treatment. Mechanism: primes immune response when safe and indicated.

7. Nutrition counseling for cancer care. Focus on adequate protein, calories, and fiber; manage nausea/constipation from therapy. Purpose: maintain strength and healing. Mechanism: supports immune cells and tissue repair.

8. Sleep optimization. Regular schedule, limit screens late evening, address steroid-related insomnia. Purpose: better energy and mood; immune support. Mechanism: normalizes circadian and stress hormones.

9. Gentle physical activity. Walking or tailored exercise as cleared by your doctor. Purpose: preserve muscle mass and mood; reduce fatigue. Mechanism: anti-inflammatory myokines, better blood flow.

10. Smoking cessation. Purpose: better ocular and systemic microcirculation; lower infection and healing complications. Mechanism: improves oxygen delivery and reduces vascular inflammation.

11. Alcohol moderation. Purpose: protect liver (important for methotrexate handling) and reduce fall risk with impaired vision. Mechanism: lowers hepatotoxic stress and sedation.

12. Stress-reduction & counseling. Psycho-oncology, mindfulness, support groups. Purpose: lower anxiety/depression that often accompany prolonged diagnostic journeys. Mechanism: reduces cortisol and improves coping.

13. Medication reconciliation & interaction checks. Review over-the-counter/herbal products before each cycle or injection. Purpose: avoid drug/supplement interactions (e.g., with high-dose methotrexate). Mechanism: pharmacist/clinician screening.

14. Sun and UV protection. Hats and sunglasses if photophobia is present. Purpose: comfort and glare reduction. Mechanism: filters light to the retina.

15. Blood pressure and glucose control. Purpose: protect retina and reduce treatment complications. Mechanism: preserves microvascular health.

16. Driving safety plan. Night-driving caution; consider DMV rules for vision. Purpose: safety for you and others. Mechanism: aligns driving demands with current visual function.

17. Work and study accommodations. Larger fonts, extra time, flexible lighting. Purpose: maintain productivity. Mechanism: ergonomics and accessibility.

18. Prevent steroid overuse before biopsy. If possible, limit steroids until samples are obtained to avoid blunting diagnostic yield. Purpose: faster, more accurate diagnosis. Mechanism: avoids steroid-induced lymphoma cell apoptosis that can mask disease. Lippincott Journals

19. Regular neuro-ophthalmic follow-up. Scheduled eye and brain checks even when vision seems stable. Purpose: catch relapses early. Mechanism: periodic imaging and exams.

20. Clinical-trial awareness. Ask your team about ongoing trials in PVRL/PCNSL (new drugs, local therapies, CAR-T). Purpose: access promising options. Mechanism: research protocols with safety oversight.

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Drug treatments

*Doses and timing below are typical literature examples, not personal medical advice. Your team will individualize them.

1. Intravitreal methotrexate (MTX) — antimetabolite
   Typical dose/schedule: 0.4 mg (400 µg/0.1 mL) per injection. A commonly used protocol is twice weekly for 4 weeks (induction), weekly for 8 weeks (consolidation), then monthly for ~9 months (maintenance), totaling ~25 injections. Purpose: clear malignant cells in the eye. Mechanism: MTX blocks folate-dependent DNA synthesis, halting fast-growing lymphoma cells. Key side effects: corneal epitheliopathy/keratopathy, cataract progression, transient IOP spikes, sterile inflammation; managed with lubrication and spacing/adjusting doses. Annals of LymphomaBioMed Central

2. Intravitreal rituximab — anti-CD20 monoclonal antibody
   Typical dose/schedule: 1 mg/0.1 mL, often weekly for 4 weeks, then monthly or PRN; sometimes alternated with MTX to reduce corneal toxicity. Purpose: kill CD20-positive lymphoma cells locally. Mechanism: antibody-dependent cellular cytotoxicity and complement activation. Key side effects: transient anterior uveitis, IOP elevations, rare sterile endophthalmitis case series reported. tvst.arvojournals.orgPubMed+1PentaVision

3. High-dose intravenous methotrexate (HD-MTX) — systemic antimetabolite
   Typical dose/schedule: commonly 3.5–8 g/m² IV every 2–3 weeks with leucovorin rescue, strict hydration and urine alkalinization; number of cycles varies. Purpose: treat/limit CNS disease and reduce brain relapse risk. Mechanism: high CNS-penetrating exposure to shut down DNA synthesis in lymphoma cells. Key side effects: kidney injury, mucositis, myelosuppression, transaminitis; requires inpatient-level monitoring. PMCEsmo Open

4. Systemic rituximab — anti-CD20 antibody
   Typical dose/schedule: 375 mg/m² IV, commonly combined with HD-MTX-based regimens. Purpose: add B-cell targeting to chemotherapy. Mechanism: same as intravitreal but systemic. Key side effects: infusion reactions, infections, late hypogammaglobulinemia. ASH Publications

5. Cytarabine (Ara-C) — antimetabolite
   Typical dose/schedule: high-dose 2 g/m² IV q12h on days 2–3 in combination regimens (center-specific). Purpose: deepen CNS control with HD-MTX regimens. Mechanism: incorporates into DNA causing chain termination. Key side effects: cytopenias, neurotoxicity at high doses. PMC

6. Temozolomide — oral alkylator (salvage/maintenance contexts)
   Typical dose/schedule: 150–200 mg/m² orally days 1–5 every 28 days (regimens vary). Purpose: salvage therapy when HD-MTX is not possible or after relapse; sometimes paired with rituximab and/or RT. Mechanism: methylates DNA at O6-guanine. Key side effects: myelosuppression, fatigue, nausea. PMCOxford Academic

7. Ibrutinib — BTK inhibitor (off-label for PCNSL/PVRL; mostly used in relapsed settings)
   Typical dose/schedule: 560 mg orally once daily (adjust per interactions/tolerability). Purpose: targeted therapy for B-cell receptor signaling. Mechanism: blocks BTK, impairing survival signaling in lymphoma cells. Key side effects: bleeding risk, atrial fibrillation, infections, diarrhea. PMC+1ScienceDirect

8. Lenalidomide — immunomodulatory drug (IMiD), often with rituximab (“R²”)
   Typical dose/schedule: 10–25 mg orally days 1–21 of 28-day cycles; rituximab added per protocol. Purpose: salvage/maintenance; can cross into CSF and may lower IL-10. Mechanism: enhances immune attack (T/NK cells), degrades IKZF1/3 in B cells. Key side effects: cytopenias, rash, thrombosis (consider prophylaxis). PMC+1Annals of Oncology

9. Intrathecal methotrexate (± cytarabine) — regional CNS therapy
   Typical dose/schedule: MTX 12 mg via lumbar puncture or Ommaya reservoir at intervals set by neuro-oncology. Purpose: treat lymphoma in spinal fluid/meninges when present. Mechanism: direct exposure to CSF-resident malignant cells. Key side effects: headache, chemical meningitis, neurotoxicity at high cumulative doses. PMC

10. Thiotepa (as part of high-dose chemo conditioning) — alkylator
    Typical use: incorporated into high-dose chemotherapy (HDC) before autologous stem-cell transplant (ASCT) in selected relapsed/fit patients; total thiotepa doses vary by center (e.g., 10–20 mg/kg) and are paired with other agents (e.g., BCNU/busulfan). Purpose: achieve deep remission before reinfusing a patient’s stem cells. Mechanism: high-intensity DNA crosslinking to eradicate resistant lymphoma. Key side effects: profound cytopenias, mucositis, infections (transplant-level care). PMC+1

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Dietary molecular/supportive supplements

Crucial note: No supplement cures PVRL. Many supplements interact with chemotherapy, immunotherapy, or warfarin. Always clear supplements with your oncology team—especially during HD-MTX, rituximab, lenalidomide, ibrutinib, or before/after transplant.

1. Vitamin D (if low). Dose: commonly 800–2000 IU/day or as prescribed to correct deficiency. Function/Mechanism: supports immune regulation, bone and muscle health; deficiency is frequent in chronic illness.

2. Calcium (diet-first, pills if deficient). Dose: diet to ~1000–1200 mg/day total intake. Mechanism: bone health during steroid use; avoid excessive doses.

3. Protein (whey/pea) supplement if intake is poor. Dose: enough to reach ~1.0–1.2 g/kg/day total protein unless restricted. Mechanism: supports healing, immune cells, and counters treatment-related weight loss.

4. Omega-3 (EPA+DHA). Dose: ~1 g/day combined EPA+DHA (with MD approval). Mechanism: anti-inflammatory lipid mediators; may help dry eye comfort and general cardiovascular support.

5. Lutein/Zeaxanthin. Dose: ~10 mg lutein + 2 mg zeaxanthin daily. Mechanism: carotenoids concentrated in macula; antioxidant support for retinal cells (adjunct only).

6. B-complex at RDA levels (avoid high “mega-dosing”). Mechanism: supports energy metabolism; caution: do not self-start folate/folinic acid during systemic HD-MTX unless your oncologist prescribes leucovorin rescue (dosing/timing is medical).

7. Prophylactic anti-nausea diet strategies (ginger tea/foods if approved, small frequent meals). Mechanism: reduces chemo-related nausea without drug interactions.

8. Soluble fiber (oats, psyllium) for bowel regularity. Mechanism: microbiome and stool consistency support during therapy.

9. Electrolyte fluids during vomiting/diarrhea (as advised). Mechanism: prevent dehydration and kidney stress (key for MTX clearance).

10. Vitamin B12 or iron only if deficient. Mechanism: corrects reversible anemia/fatigue drivers; avoid unnecessary supplements without lab confirmation.

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Regenerative” drugs & cellular approaches

There are no proven “stem-cell drugs” for PVRL. Evidence-based immune-based options are mainly targeted agents and antibodies; cellular therapies are reserved for clinical trials or selected relapsed cases.

1. Rituximab (anti-CD20 antibody). Dose: 375 mg/m² IV (systemic) or 1 mg/0.1 mL intravitreal. Function/Mechanism: flags B-cells for immune clearance. Notes: cornerstone drug in many regimens. ASH Publicationstvst.arvojournals.org

2. Lenalidomide (IMiD). Dose: 10–25 mg d1–21 q28d (per protocol). Function/Mechanism: boosts T/NK-cell activity and directly stresses lymphoma cells; can reduce IL-10. Notes: used as maintenance or salvage; VTE prophylaxis often considered. PMC

3. Ibrutinib (BTK inhibitor). Dose: 560 mg daily. Function/Mechanism: shuts down B-cell receptor signaling. Notes: active in relapsed PCNSL/PVRL in studies; drug interactions and bleeding risk need management. ScienceDirect

4. PD-1 inhibitors (e.g., nivolumab, pembrolizumab). Dose: nivolumab 240 mg q2w/480 mg q4w per study; pembrolizumab regimens vary. Function/Mechanism: unleashes T-cells against tumor by blocking PD-1. Notes: emerging data in relapsed CNS lymphomas; use mainly in trials/selected salvage. ASH Publications

5. Autologous hematopoietic stem-cell transplantation (ASCT) after high-dose chemotherapy (HDC) (includes thiotepa-based conditioning). Function/Mechanism: uses your own stem cells to restore marrow after intensive chemo that aims for deep remission. Notes: for fit, relapsed/selected patients under transplant teams. PMC

6. CAR-T cell therapy (anti-CD19 products) — investigational/expanding access in CNS lymphoma. Function/Mechanism: your T-cells engineered to hunt CD19-positive lymphoma. Notes: Emerging series suggest feasible and active even with CNS disease; still specialized with unique toxicities (CRS/ICANS). PMC+1

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Procedures / “surgeries

1. Diagnostic pars plana vitrectomy with vitreous biopsy. Purpose: obtain enough cells for cytology, flow cytometry, IL-10/IL-6 ratio, and genetic tests (e.g., MYD88 L265P). Mechanism: removes vitreous gel for lab analysis; often improves vision by clearing dense haze. MDPIPMC

2. Targeted retinal/chorioretinal biopsy (rare). Purpose: when vitreous sampling is inadequate yet suspicion remains high. Mechanism: small tissue sample from infiltrated retina/choroid. EyeWiki

3. Intravitreal injection procedures (MTX/rituximab). Purpose: deliver drug directly to eye lymphoma. Mechanism: high local concentration with limited systemic exposure. Annals of Lymphoma

4. External-beam ocular radiotherapy. Purpose: sterilize intraocular lymphoma, especially if injections are not tolerated or as consolidation. Mechanism: DNA damage in tumor cells; common total doses ~25–36 Gy with modern planning to limit toxicity. PMC+1

5. Ommaya reservoir placement (for intrathecal therapy). Purpose: reliable access for methotrexate/cytarabine when CSF involvement exists. Mechanism: a small dome under the scalp connected to the ventricles to deliver drugs into CSF. PMC

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Prevention

• There is no known way to prevent PVRL at the population level. Focus is on early recognition and preventing complications.

1. Seek a retina/uveitis specialist when “uveitis” does not respond to steroids or keeps coming back. JAMA Network

2. Avoid or minimize steroids before diagnostic sampling, when safely possible. Lippincott Journals

3. Keep all follow-up imaging (eyes and brain) to catch progression early. EyeWiki

4. Protect kidney function during systemic MTX (hydration, alkalinization per team). PMC

5. Keep vaccinations up to date (non-live when appropriate) to reduce infections during therapy.

6. Practice ocular hygiene to lower post-injection infection risk.

7. Manage comorbidities (BP, diabetes) to preserve retinal health.

8. Stop smoking and limit alcohol to improve healing.

9. Discuss fertility and contraception before systemic therapy if relevant.

10. Consider clinical trials early when available.

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When to see a doctor urgently

• Sudden worsening of vision, new floaters, flashes, eye pain, or a curtain-like shadow.

• Persistent “uveitis” that does not improve with steroids or keeps relapsing. JAMA Network

• Severe headache, confusion, weakness, seizures, or any new neurological symptom.

• Fever, signs of infection, bleeding, or severe fatigue during therapy.

• Eye redness/pain after an injection (to exclude infection).

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What to eat—and what to avoid

What to eat

1. Protein with every meal (eggs, fish, poultry, legumes, dairy/soy) to maintain strength.

2. Plenty of fruits/vegetables of varied colors for fiber and micronutrients.

3. Whole grains for energy and bowel regularity.

4. Healthy fats (olive oil, nuts, seeds; omega-3 fish).

5. Hydration (water, oral rehydration solutions during vomiting/diarrhea).

6. Calcium & vitamin D sources (dairy/fortified foods, safe sunlight) if cleared.

7. Small, frequent meals on treatment days to counter nausea.

8. Foods gentle on the stomach (bananas, rice, applesauce, toast, yogurt) during flares.

9. High-fiber foods (oats, psyllium, legumes) when constipated from meds (unless your team says otherwise).

10. Food safety focus: well-washed produce, fully cooked proteins; avoid raw/undercooked foods during neutropenia.

What to avoid

1. Excess alcohol (hepatotoxicity with MTX; fall risk with poor vision).

2. High-dose herbal/antioxidant supplements during chemo/radiation unless your oncologist approves (possible interactions).

3. NSAIDs without checking (bleeding risk with ibrutinib/low platelets; kidney stress with MTX).

4. Grapefruit/Seville orange with certain targeted drugs (CYP3A interactions—ask your team).

5. Raw seafood/unpasteurized products if neutropenic.

6. Megadose folate/folinic acid unless prescribed as leucovorin with systemic HD-MTX.

7. High-sugar beverages if steroids raise blood sugar—prefer water or unsweetened options.

8. Caffeine excess if it worsens tremor/palpitations during therapy.

9. Very salty foods if you have steroid-related fluid retention.

10. Unvetted “immune boosters.” Stick to evidence-based care; discuss any product first.

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Frequently asked questions (FAQs)

1) Is PVRL the same as uveitis?
No. It mimics uveitis but is a lymphoma. If “uveitis” keeps returning or does not respond to steroids, doctors consider PVRL and may biopsy. JAMA Network

2) How is PVRL confirmed?
By vitreous biopsy with cytology/flow, IL-10:IL-6 ratio, and molecular tests like MYD88 L265P; imaging and brain MRI complete the work-up. MDPIPMC

3) What is the role of IL-10:IL-6?
A ratio >1 strongly points toward lymphoma rather than uveitis, though it’s not perfect and can be altered by steroids. MDPIScienceDirect

4) Why avoid steroids before biopsy?
They can kill or shrink lymphoma cells temporarily, causing false-negative tests and delayed diagnosis. Lippincott Journals

5) What are the main eye-directed treatments?
Intravitreal methotrexate and/or intravitreal rituximab injections; sometimes eye radiotherapy. Annals of Lymphomatvst.arvojournals.org

6) What about brain involvement?
Doctors use high-dose IV methotrexate-based regimens (often with rituximab and/or cytarabine) to treat or prevent CNS disease. PMC

7) Are intravitreal injections safe?
They are widely used. Common issues include short-term pressure rises and surface irritation; infection is rare but serious; rituximab has rare sterile endophthalmitis reports. tvst.arvojournals.orgPubMed

8) Is ocular radiotherapy effective?
Modern 25–36 Gy courses give high local control with careful planning to limit retinopathy and cataract risks. PMC

9) Will I need both local and systemic treatment?
Often yes, depending on staging and risk. Your team balances eye control and CNS protection based on your case. EyeWiki

10) Can PVRL come back?
Relapses can occur in the eyes or CNS; scheduled follow-ups aim to catch them early. ASH Publications

11) Are “immune boosters” helpful?
Not proven for PVRL; some “natural” products interact with treatment. Discuss every supplement with your team.

12) Do I need to change my diet?
Use a cancer-smart nutrition plan (adequate protein, hydration, safe food handling). Avoid alcohol excess and unapproved supplements.

13) Is transplant (ASCT) or CAR-T an option?
In relapsed/selected cases and specialized centers, ASCT after high-dose chemo or CAR-T may be considered; these are not first-line for most people with isolated eye disease. PMC+1

14) What about PD-1 immunotherapy?
Small studies in relapsed CNS lymphomas show activity; use is mainly in trials or selected salvage settings. ASH Publications

15) What should I tell new doctors?
That you have/are being worked up for primary vitreoretinal lymphoma, any brain/CNS findings, and exact drugs/doses you received (intravitreal and systemic).

Disclaimer: Each person’s journey is unique, treatment plan, life style, food habit, hormonal condition, immune system, chronic disease condition, geological location, weather and previous medical  history is also unique. So always seek the best advice from a qualified medical professional or health care provider before trying any treatments to ensure to find out the best plan for you. This guide is for general information and educational purposes only. Regular check-ups and awareness can help to manage and prevent complications associated with these diseases conditions. If you or someone are suffering from this disease condition bookmark this website or share with someone who might find it useful! Boost your knowledge and stay ahead in your health journey. We always try to ensure that the content is regularly updated to reflect the latest medical research and treatment options. Thank you for giving your valuable time to read the article.

The article is written by Team RxHarun and reviewed by the Rx Editorial Board Members

Last Updated: August 23, 2025.