Uveal Lymphoma 

Uveal lymphoma is a cancer of lymphocytes (a type of white blood cell) that grows inside the uvea of the eye. The uvea is the middle layer of the eyeball and has three parts: the iris (the colored part in front), the ciliary body (a ring of muscle and tissue that helps the eye focus), and the choroid (a layer rich in blood vessels that feeds the retina). When lymphoma cells enter or arise in these uveal tissues, they can thicken the choroid, change how light passes through the eye, and disturb the retina. This can cause blurred vision, floaters, or patchy loss of sight. Doctors call this group of problems intraocular lymphoma when the disease is inside the eye. Uveal lymphoma usually affects the choroid, and many cases are a slow-growing B-cell tumor called extranodal marginal zone lymphoma or MALT lymphoma. Some cases, especially when the vitreous (the gel in the middle of the eye) and retina are involved, are more aggressive and are called vitreoretinal lymphoma, most often a diffuse large B-cell lymphoma. These patterns matter because they look different on eye imaging tests and need different tests to confirm the diagnosis. AAO JournalPubMed+1EyeWiki

Uveal lymphoma is rare. It can appear by itself in the eye (primary disease) or appear when someone already has lymphoma elsewhere in the body and the cancer spreads to the uvea (secondary disease). Choroidal involvement often looks like diffuse thickening under the retina rather than a single round mass, and this is an important clue during ultrasound and optical coherence tomography (OCT) imaging. Because the disease is rare, doctors rely on a combination of symptoms, eye examination, special eye imaging, and sometimes biopsy (taking tissue or fluid to look at cells) to make the diagnosis. Measuring certain immune signals in eye fluid, such as the ratio of interleukin-10 (IL-10) to interleukin-6 (IL-6), and testing for MYD88 gene mutations can help confirm lymphoma that involves the vitreous and retina. PMC+2PMC+2Annals of Lymphoma

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Types of Uveal Lymphoma

1) Primary uveal (choroidal) marginal zone/MALT lymphoma.
This is the most common uveal type. “Primary” means it starts in the eye rather than spreading from somewhere else. “Marginal zone/MALT” means the cancer cells look and behave like B-cells that normally live in mucosa-associated lymphoid tissue. In the eye, this often shows up as diffuse, sheet-like thickening of the choroid with subtle yellow-orange patches under the retina. It often grows slowly. AAO JournalPubMed

2) Secondary uveal involvement from systemic lymphoma.
This means a person already has lymphoma in another organ (like lymph nodes, bone marrow, or other tissues) and the cancer spreads to the choroid. Secondary disease is more likely to be in both eyes and can be more aggressive than primary uveal MALT lymphoma. PMC

3) Vitreoretinal lymphoma (VRL) with uveal extension.
VRL mainly involves the vitreous and retina, often as a diffuse large B-cell lymphoma (DLBCL). It can touch the choroid and uvea as it progresses. Doctors look for high IL-10 relative to IL-6 in eye fluid and may test for MYD88 L265P mutation, which supports the diagnosis. VRL is closely linked to central nervous system (CNS) lymphoma and needs a careful brain evaluation. PMCEyeWiki

4) Other rare B-cell types in the uvea.
Uncommonly, the uvea can be involved by follicular lymphoma, mantle cell lymphoma, or other B-cell subtypes. These are unusual but possible and are identified by immunohistochemistry, flow cytometry, and molecular tests on tissue or eye fluid. PubMed

5) Overlap with ocular adnexal lymphoma.
Lymphoma can arise in tissues around the eye (ocular adnexa)—like the conjunctiva, eyelids, or orbit—and extend to the uvea. Ocular adnexal lymphoma is also commonly a MALT lymphoma. There is overlap between adnexal and uveal involvement, so careful imaging of both the eyeball and the orbit is important. PubMedPMC

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Causes and Risk Contexts

Because uveal lymphoma is rare, many “causes” are better described as risk contexts, origins, or known associations that make uveal involvement by lymphoma more likely. Some are strong and specific (like VRL biology), while others are general lymphoma risk factors with limited direct evidence for the uvea. I clearly label them and keep language simple.

1. Primary MALT biology in the choroid.
   Some uveal lymphomas start in the choroid as marginal zone/MALT lymphoma. This is an intrinsic origin in eye tissue rather than spread from elsewhere. AAO JournalPubMed

2. Secondary spread from systemic lymphoma.
   Systemic lymphoma can seed the choroid, causing uveal disease later in the illness. This is a common route for uveal involvement. PMC

3. Vitreoretinal lymphoma patterns.
   VRL is an aggressive B-cell lymphoma of the vitreous/retina that can extend to uveal layers. It often carries MYD88 mutations and shows high IL-10 in eye fluid. PMC

4. Age (older adults).
   Most patients are middle-aged or older, often in the 50s to 70s, which reflects the general age pattern of many B-cell lymphomas. AAO JournalWebEye

5. Male sex slightly more represented in some series.
   Several case series of uveal lymphoma showed a male majority, suggesting a modest sex tilt. AAO Journal

6. Chronic immune stimulation (general lymphoma concept).
   Long-standing immune stimulation is a known driver for MALT lymphomas at many sites and may contribute to ocular MALT biology, though direct proof for the choroid is limited. NCBI

7. Autoimmune disease associations (general marginal zone context).
   Autoimmune conditions like Sjögren syndrome and Hashimoto thyroiditis are linked to MALT lymphomas at other sites. This is a background risk concept rather than a uvea-specific proof. NCBI

8. Infections driving MALT at other sites (background concept).
   Some MALT lymphomas are infection-related (e.g., H. pylori in stomach, C. psittaci reported for ocular adnexa). For uvea, infection links are not well established, but clinicians consider this general MALT framework. TRI Kobe

9. Immunosuppression (including post-transplant).
   Weak immune surveillance (e.g., post-transplant lymphoproliferative disorders) can increase lymphoma risk overall, which may include ocular sites. This is a general lymphoma risk applied to the eye. Gluetacs

10. Hepatitis C virus (HCV) (marginal zone association).
    HCV is linked to some marginal zone lymphomas. It is a systemic association that informs risk thinking even if not proven for the uvea specifically. NCBI

11. Pre-existing ocular adnexal MALT lymphoma.
    Lymphoma in conjunctiva, orbit, or eyelid can extend inward to the uvea, creating apparent uveal disease. PMC

12. History of other extranodal MALT lymphomas.
    Patients with MALT lymphoma in another organ may later have ocular involvement, including the uvea. TRI Kobe

13. Diffuse large B-cell lymphoma elsewhere.
    Patients with DLBCL can have ocular spread, including the choroid and retina. This is a recognized route for secondary uveal disease. EyeWiki

14. Prior radiation/chemotherapy exposure (general oncologic risk).
    Past cancer treatments can alter immune and tissue environments in ways that raise lymphoma risk in general. Evidence for uveal-specific risk is limited, but clinicians consider the background risk. (General concept supported by oncology practice guidelines for systemic workup.) Gluetacs

15. Family history of lymphoma (general risk).
    A family history may modestly increase overall lymphoma risk, which could include ocular sites, though uvea-specific data are sparse. (General lymphoma risk context.) NCCN

16. Older immune system (immunosenescence).
    With age, immune control of abnormal B-cells weakens. Many patients with uveal lymphoma are older, matching this age-linked biology. AAO Journal

17. Systemic “MALT-prone” states (e.g., chronic antigen exposure).
    People with repeated antigen exposure in mucosal tissues may be at risk for MALT processes. It is a framework rather than a uvea-specific proof. NCBI

18. CNS lymphoma with ocular involvement.
    Patients with primary CNS lymphoma can develop ocular/vitreoretinal involvement that may extend to uveal tissues, especially with VRL patterns. Rare Disease Advisor

19. Monoclonal gammopathies/Waldenström macroglobulinemia (rare contexts).
    Certain B-cell disorders can have ocular involvement; direct uveal infiltration is uncommon, but relevant as a differential route. (General B-cell lymphoma context.) Gluetacs

20. No identifiable trigger.
    In many people, no clear cause is found. The disease appears sporadically, as seen in multiple case series of uveal lymphoma. AAO Journal

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Common Symptoms

1. Blurred vision.
   Vision becomes fuzzy or less sharp because lymphoma thickens the choroid and disturbs the retina’s smooth contour, which is essential for fine focus. PMC

2. Floaters.
   Small spots or cobwebs drift in the line of sight when lymphoma cells or inflammation are present in the vitreous gel. This is typical of vitreoretinal lymphoma, which can also touch the uvea. EyeWiki

3. Patchy blind spots (scotomas).
   Areas of vision are missing because parts of the retina do not receive normal support from the thickened choroid underneath. AAO Journal

4. Distorted vision (metamorphopsia).
   Straight lines may look bent when retinal layers are lifted or wrinkled by choroidal swelling or subretinal fluid. AAO Journal

5. Trouble seeing in dim light (night vision problems).
   Rod cells struggle when the choroid is thick and nutrition to the outer retina is uneven. AAO Journal

6. Photopsia (flashes of light).
   Retinal irritation from adjacent lymphoma changes can create brief light flashes. EyeWiki

7. Color vision changes.
   Colors can look washed out when the macula is affected by subretinal fluid or bulging of the layers. AAO Journal

8. Reduced contrast sensitivity.
   Fine detail in low-contrast scenes becomes hard to see if macular architecture is disturbed. AAO Journal

9. Visual field loss (peripheral or central).
   If lymphoma is diffuse, the field of vision may shrink or develop central gaps. AAO Journal

10. Eye redness is uncommon.
    Most patients with choroidal lymphoma do not have red, painful eyes; symptoms are often subtle and painless. Pain suggests another diagnosis. PMC

11. Mild eye discomfort or pressure sense.
    Some patients feel vague pressure if the tumor affects fluid flow or ciliary body function. AAO Journal

12. Double vision (rare in pure uveal disease).
    Diplopia can occur if adnexal/orbital lymphoma coexists and moves the eye. PMC

13. Worsening vision despite steroids.
    Temporary improvement with steroids can mislead because steroids quiet inflammation but do not treat lymphoma, so vision may worsen again. This “steroid-responsive but relapsing” pattern is a classic masquerade sign. PMC

14. Systemic “B” symptoms when disease is secondary.
    Fevers, night sweats, weight loss point to systemic lymphoma that may also involve the uvea. PMC

15. Symptoms in both eyes more often in secondary disease.
    Both eyes being involved raises suspicion for secondary lymphoma rather than purely primary uveal MALT. PMC

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Diagnostic Tests

I group the tests into Physical Exam, Manual/In-office functional tests, Lab & Pathological tests, Electrodiagnostic tests, and Imaging tests. In practice, doctors choose a mix of these based on symptoms, eye exam, and whether there is known or suspected lymphoma elsewhere.

A) Physical Examination (whole-patient and eye-focused)

1) Comprehensive medical history and full physical exam.
Doctors ask about vision changes, timeline, prior cancers, autoimmune disease, infections, and medications. They examine the lymph nodes, skin, abdomen, and nervous system to look for signs of systemic lymphoma. This is also part of standard NCCN workup for lymphoma. PMC

2) External eye and orbit inspection.
The doctor looks for eyelid swelling, proptosis (eye bulging), or conjunctival lesions that may suggest ocular adnexal lymphoma, which sometimes coexists with uveal involvement. PMC

3) Cranial nerve and neuro-ophthalmic screening.
Tests of eye movements, pupil reactions, and facial sensation help identify spread to the orbit or CNS, which can guide imaging and staging. EyeWiki

4) General performance status assessment.
Clinicians rate overall function because it influences the staging plan and future treatment choices; this is recommended for lymphoma evaluations. PMC

5) Systemic symptom review (B symptoms).
Asking about fevers, night sweats, weight loss is important to spot systemic disease, which changes the urgency and scope of workup. PMC

B) Manual and In-Office Functional Tests (simple, non-invasive)

6) Best-corrected visual acuity and pinhole test.
Measures how clearly a person sees and whether blur is optical (improved by pinhole) or retinal (not improved). In uveal lymphoma, blur often persists despite pinhole. (Clinical standard; supported by case series describing visual loss patterns.) PMC

7) Amsler grid and metamorphopsia check.
A simple square grid helps detect wavy lines or missing boxes when the macula is disturbed by subretinal fluid or choroidal bulging. AAO Journal

8) Color vision testing (Ishihara or similar).
Subtle color desaturation can appear when the macula is affected. Tracking color vision helps monitor function over time. AAO Journal

9) Confrontation visual fields.
A bedside check for blind spots and field loss; it is quick and flags the need for formal perimetry if defects are found. AAO Journal

10) Intraocular pressure by applanation tonometry.
Measures eye pressure; abnormal pressure does not diagnose lymphoma but can change if the ciliary body is affected or if inflammation alters fluid outflow, guiding supportive care while diagnosis proceeds. (Clinical practice context.) AAO Journal

C) Laboratory & Pathological Tests (definitive diagnosis and staging)

11) Complete blood count (CBC) and serum LDH.
CBC checks for anemia or abnormal white cells; LDH is a general tumor activity marker used in lymphoma staging. These are standard lymphoma labs per NCCN-style workups. PMC

12) Viral serology (HIV, hepatitis B/C).
Important for lymphoma care planning and to identify co-infections that change management and safety of rituximab or other treatments later; part of standard staging labs. PMC

13) Beta-2 microglobulin and basic metabolic panel.
General lymphoma markers and organ function tests that help risk-stratify and plan imaging or biopsy. (Guideline workup context.) Gluetacs

14) Diagnostic vitreous/aqueous tap for cytology.
A small sample of eye fluid is taken to look for lymphoma cells under the microscope. Sometimes a retinal or choroidal biopsy is needed if taps are inconclusive. PMC

15) Flow cytometry and immunohistochemistry (IHC).
These tests tell whether cells are B-cells, what markers they carry (CD20, CD79a, BCL2, etc.), and help classify MALT vs DLBCL. This is essential for correct subtype labeling. AAO Journal

16) IGH gene rearrangement studies (clonality).
PCR testing looks for clonal B-cell populations, supporting lymphoma rather than inflammation. This adds confidence when cytology is scant. AAO Journal

17) IL-10 and IL-6 levels in intraocular fluid (IL-10:IL-6 ratio).
An IL-10:IL-6 ratio > 1 in eye fluid strongly suggests vitreoretinal lymphoma. It is not perfect, but it is a very useful, minimally invasive marker. PMCAnnals of Lymphoma

18) MYD88 L265P mutation testing (molecular).
This mutation supports the diagnosis of DLBCL-type VRL. Detecting it in eye fluid or tissue strongly strengthens the case for lymphoma. PMC

19) Cerebrospinal fluid (CSF) cytology when CNS involvement is suspected.
Because VRL often coexists with brain lymphoma, doctors may sample CSF for lymphoma cells as part of staging when it is safe and appropriate. Rare Disease Advisor

20) Bone marrow biopsy (selected cases).
If systemic staging suggests a need, a bone marrow biopsy can complete lymphoma staging, especially in marginal zone or DLBCL contexts. PMC

D) Electrodiagnostic Tests (measure retinal function)

21) Full-field electroretinography (ERG).
ERG measures the electrical responses of the whole retina to light. In uveal lymphoma that affects the outer retina (via choroidal changes) or in VRL, ERG can show reduced function, helping separate retinal disease from pure optical blur. (Electrodiagnostic role described in intraocular lymphoma reviews.) PMC

22) Multifocal ERG (mfERG).
mfERG maps the central retina function in many small zones. It helps locate and track macular dysfunction caused by subretinal fluid or choroidal thickening. (Functional testing context in VRL/retinal disease.) PMC

23) Electro-oculography (EOG).
EOG evaluates the retinal pigment epithelium (RPE) health. When choroidal lymphoma disturbs the RPE/photoreceptor interface, EOG changes can appear. (Supportive functional tool.) PMC

24) Visual evoked potentials (VEP).
VEP checks the optic nerve and visual pathway function. It can help when there is suspicion of CNS involvement along with ocular disease. EyeWiki

E) Imaging Tests (eye imaging and systemic staging)

25) Optical coherence tomography (OCT).
OCT is a light-based scanner that shows cross-sections of the retina and choroid. In uveal lymphoma, OCT often shows choroidal thickening, undulating RPE/Bruch’s lines, and subretinal fluid. Enhanced-depth or swept-source OCT can better show the choroid. AAO Journal

26) Fundus autofluorescence (FAF).
FAF maps natural signals from the RPE. Patchy bright or dark areas can outline stress or damage overlying choroidal lymphoma. It helps track change over time. AAO Journal

27) Fluorescein angiography (FA).
FA shows retinal circulation. In choroidal lymphoma, FA may reveal leakage or mottled patterns over lesions, helping distinguish it from inflammatory choroiditis or melanoma. AAO Journal

28) Indocyanine green angiography (ICG).
ICG highlights the choroidal circulation. Hypocyanescent (dark) spots or plaques can mark choroidal lymphoma and show its true extent better than FA. AAO Journal

29) B-scan ocular ultrasonography.
Ultrasound uses sound waves to see through opaque media. In choroidal lymphoma, classic findings are diffuse choroidal thickening and a smooth, shallow elevation rather than a dome-shaped mass. Ultrasound is highly useful when the view is cloudy. PMC+1

30) Ultrasound biomicroscopy (UBM).
UBM is high-frequency ultrasound for the front of the eye. It helps when the ciliary body is involved and can show thickening or masses hidden behind the iris. AAO Journal

31) Wide-field color fundus photography.
Photographs document baseline lesions, subretinal fluid, and choroidal folds so changes can be tracked over months. This supports clinical correlation with OCT and angiography. AAO Journal

32) MRI of the orbits and brain with contrast.
MRI shows the eyeball, choroid, optic nerve, orbit, and brain. It helps assess adnexal involvement and screens for CNS disease, which is vital in VRL. Diffusion-weighted MRI can highlight lymphoma tissue. EyeWiki

33) CT of the orbits/sinuses (selected).
CT is helpful if there is bone, sinus, or calcification concern in the orbit and to plan biopsies in adnexal disease that may extend toward the uvea. PMC

34) Whole-body PET-CT for staging.
PET-CT looks for active lymphoma throughout the body and determines if uveal disease is primary or part of systemic lymphoma. It guides biopsy sites and overall staging. PMC

35) Chest/abdomen/pelvis CT for systemic staging.
If PET-CT is unavailable or to complement PET, CT scans check for lymph nodes and organ involvement to complete the picture of disease spread. PMC

36) Targeted ultrasound of lymph nodes or abdomen (selected).
When imaging suggests abnormal nodes or organs, targeted ultrasound helps sample (biopsy) safely. This is part of a rational, stepwise staging plan. PMC

37) Serial OCT and imaging follow-up.
Repeated imaging over time shows whether lesions grow or fluid changes, which is vital in slowly progressive choroidal MALT or when diagnosis is uncertain and biopsy is pending. AAO Journal

38) Formal automated perimetry (visual field test).
A computerized test maps sensitivity at many points. It detects and tracks small scotomas caused by choroidal thickening under the macula or near the optic nerve. AAO Journal

39) OCT-angiography (OCT-A).
OCT-A shows capillary flow without dye. It can demonstrate choriocapillaris flow voids or distortions overlying lymphoma-thickened choroid. It is an adjunct to FA/ICG. AAO Journal

40) Multimodal imaging synthesis (combined read).
Combining OCT + FA + ICG + ultrasound + MRI gives a full picture: where the disease sits, how active it is, and whether there is extraocular spread. Studies show that combined imaging plus aqueous testing improves diagnostic confidence, especially for choroidal lymphoma. PMC

Non-pharmacological treatments (therapies & others)

These include eye-directed procedures and whole-person supportive care. For each: Description – Purpose – Mechanism

1. Observation (“watchful waiting”).
   What: Close follow-up when disease is tiny, stable, and symptoms are minimal.
   Why: Avoid overtreatment; treat if growth or symptoms occur.
   How: Serial exams/OCT/MRI track stability.

2. External-beam radiation therapy (EBRT).
   What: Precisely aimed radiation to the uvea/orbit.
   Why: First-line for localized uveal MALT lymphoma with high local control.
   How: DNA damage kills lymphoma cells while sparing nearby tissue via planning.

3. Plaque brachytherapy (surgical placement, then removal).
   What: A small radioactive “plaque” sutured to the sclera over the lesion for days.
   Why: Delivers high local dose with rapid fall-off.
   How: Radiation seeds (I-125, Ru-106) kill tumor cells.

4. Photodynamic therapy (PDT).
   What: IV light-activated drug (e.g., verteporfin) + targeted laser in the eye.
   Why: Selected small choroidal lesions or fluid control when radiation is undesirable.
   How: Activated drug generates reactive oxygen species, damaging neovasculature/tumor micro-vessels.

5. Focal thermal laser (rarely used).
   What: Gentle photocoagulation for specific tiny foci.
   Why: Niche role; sometimes adjunctive.
   How: Heat causes local tumor micro-destruction; use is limited.

6. Pars plana vitrectomy (PPV) for diagnosis/clarity.
   What: Microsurgery to remove vitreous haze, obtain cells, improve imaging.
   Why: Aids diagnosis; can improve vision if floaters dense.
   How: Tiny instruments remove vitreous; specimens sent for cytology/flow.

7. Low-vision rehabilitation.
   What: Magnifiers, contrast tools, lighting, training.
   Why: Preserves function and independence despite visual deficits.
   How: Optics and coaching maximize remaining vision.

8. Protective eyewear & blue-light hygiene.
   What: Safety glasses, glare control, screen habits.
   Why: Reduces strain and incidental injury during therapy.
   How: Filters and physical barriers.

9. Smoking cessation counseling.
   What: Structured quit plans.
   Why: Smoking worsens vascular health and cancer outcomes.
   How: Behavioral support improves quit rates.

10. Nutrition counseling (anti-inflammatory pattern).
    What: Diet rich in vegetables, fruits, whole grains, lean proteins, healthy fats.
    Why: Supports healing, maintains weight/muscle during therapy.
    How: Adequate macro/micronutrients reduce catabolism and fatigue.

11. Exercise therapy (tailored).
    What: Walking/resistance per tolerance.
    Why: Preserves muscle, mood, and treatment tolerance.
    How: Boosts mitochondrial and cardiovascular function.

12. Sleep optimization.
    What: Regular schedule, dark cool room, limit late caffeine.
    Why: Improves immune signaling and daytime energy.
    How: Restorative sleep modulates cortisol and inflammation.

13. Psychosocial support / counseling.
    What: Coping skills, anxiety/depression care.
    Why: Cancer distress is common; support improves adherence and quality of life.
    How: Cognitive-behavioral strategies, peer groups.

14. Vaccination review (non-live as appropriate).
    What: Flu, COVID-19, pneumococcal, etc., timed around therapy.
    Why: Prevents infections that interrupt treatment.
    How: Immune priming reduces severe infections.

15. Infection-prevention routines.
    What: Hand hygiene, prompt fever checks, dental care.
    Why: Lower infection risks during/after therapy.
    How: Reduces pathogen exposure and entry.

16. Medication review (polypharmacy check).
    What: Reconcile eye drops, steroids, anticoagulants, supplements.
    Why: Avoid drug interactions around procedures and chemo.
    How: Pharmacist/clinician rationalizes regimen.

17. Blood pressure, glucose, and lipid control.
    What: Manage comorbidities.
    Why: Lowers radiation/chemo vascular risks and supports ocular perfusion.
    How: Lifestyle ± meds.

18. Dry eye care if needed.
    What: Lubricants, warm compresses, lid hygiene.
    Why: Comfort and vision stability, especially with radiation.
    How: Stabilizes tear film and ocular surface.

19. Work and driving adjustments.
    What: Lighting, contrast, breaks, occupational therapy input.
    Why: Keeps activities safe during treatment.
    How: Environmental modifications.

20. Scheduled surveillance.
    What: Regular eye exams and imaging; systemic staging as advised.
    Why: Detects recurrence, progression, or CNS involvement early.
    How: Timed OCT/MRI/clinical checks.

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Drug treatments

Doses are typical examples; clinicians personalize based on subtype, size, vision, age, kidneys, liver, and co-illnesses.

1. Rituximab (anti-CD20 monoclonal antibody).
   Dose: 375 mg/m² IV weekly ×4 (induction), with maintenance schedules in some cases; intravitreal 1 mg/0.1 mL for VRL/ocular disease in selected protocols.
   Purpose: Targets B-cell lymphomas (CD20+).
   Mechanism: Antibody-dependent B-cell killing.
   Side effects: Infusion reactions, infections, hypogammaglobulinemia, rare PML.

2. Chlorambucil (alkylating agent).
   Dose: 6–10 mg orally daily (varies; finite cycles).
   Purpose: Indolent MALT control, sometimes elderly/low-burden cases.
   Mechanism: DNA cross-linking → apoptosis.
   Side effects: Myelosuppression, nausea, secondary malignancy risk (long term).

3. Bendamustine + Rituximab (BR).
   Dose: Bendamustine 90 mg/m² IV Days 1–2 + Rituximab 375 mg/m² Day 1, q28d ×4–6 cycles.
   Purpose: Effective for marginal zone and other indolent B-cell lymphomas.
   Mechanism: Alkylator-purine-like action + anti-CD20.
   Side effects: Neutropenia, infections, rash, nausea; PJP prophylaxis often considered.

4. R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine, prednisone).
   Dose: Standard q21-day cycles as per guidelines.
   Purpose: For aggressive B-cell subtypes or VRL with systemic disease.
   Mechanism: Multi-agent cytotoxic + anti-CD20 synergy.
   Side effects: Myelosuppression, cardiotoxicity (doxorubicin), neuropathy (vincristine), infections.

5. High-dose Methotrexate (HD-MTX) ± rituximab/temozolomide (CNS-directed).
   Dose: Protocolized IV doses with leucovorin rescue; intravitreal MTX 400 µg/0.1 mL on loading/maintenance schedules for VRL.
   Purpose: Retina/CNS-linked lymphoma control.
   Mechanism: Folate antagonist inhibiting DNA synthesis.
   Side effects: Mucositis, renal/hepatic toxicity; strict monitoring required.

6. Ibrutinib (BTK inhibitor).
   Dose: 560 mg orally daily (varies by indication).
   Purpose: Relapsed/refractory marginal zone or other B-cell lymphomas.
   Mechanism: Blocks B-cell receptor signaling.
   Side effects: Atrial fibrillation, bleeding, diarrhea, infections, hypertension.

7. Acalabrutinib or Zanubrutinib (next-gen BTK inhibitors).
   Dose: Acalabrutinib 100 mg twice daily; Zanubrutinib 160 mg twice daily (per label).
   Purpose: Alternative BTK blockade with different side-effect profiles.
   Mechanism: More selective BTK inhibition.
   Side effects: Headache, cytopenias, infections; monitor interactions.

8. Lenalidomide + Rituximab (“R²”).
   Dose: Lenalidomide ~20 mg Days 1–21 q28d + Rituximab per protocol.
   Purpose: Immunomodulatory option for indolent B-cell lymphomas.
   Mechanism: T-cell/NK activation, anti-angiogenic effects.
   Side effects: Cytopenias, rash, thrombosis (use VTE prophylaxis as indicated), teratogenicity.

9. PI3K pathway inhibitors (e.g., Copanlisib IV; Idelalisib PO — use evolving).
   Dose: Copanlisib 60 mg IV Days 1, 8, 15 q28d; Idelalisib 150 mg twice daily (labels vary; safety updates frequent).
   Purpose: Relapsed indolent lymphomas; use guided by current approvals/safety notices.
   Mechanism: Blocks survival signaling downstream of BCR.
   Side effects: Colitis, hepatotoxicity, infections; careful selection and monitoring essential.

10. Systemic corticosteroids (e.g., Prednisone).
    Dose: Short courses at clinician discretion.
    Purpose: Reduce inflammatory component and edema; not curative for lymphoma.
    Mechanism: Broad anti-inflammatory effects.
    Side effects: Glucose rise, mood changes, cataract/glaucoma with chronic use.

Other eye-directed drugs (often adjunctive in VRL): Intravitreal rituximab and intravitreal methotrexate are used on specialized schedules to directly suppress intraocular lymphoma cells.

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Dietary “molecular” supplements

Always review supplements with your oncology/eye team to avoid interactions.

1. Vitamin D3 (1,000–2,000 IU/day). Supports immune balance and bone health; acts via vitamin-D receptor gene signaling.

2. Omega-3 (EPA+DHA) (1–2 g/day). Anti-inflammatory lipid mediators; helps tear film and systemic inflammation.

3. Curcumin (with piperine) (500–1,000 mg/day). Modulates NF-κB and cytokines; low bioavailability—use standardized forms.

4. Green tea extract (EGCG) (400–800 mg/day). Antioxidant/AMPK effects; may reduce oxidative stress.

5. Selenium (100–200 µg/day). Antioxidant selenoproteins; avoid excess.

6. Melatonin (3 mg at night). Sleep quality; antioxidant signaling.

7. Probiotics (per label). Gut-immune crosstalk; may reduce antibiotic-associated diarrhea.

8. Ginger extract (1–2 g/day). Can ease nausea and mild inflammation.

9. Medicinal mushroom β-glucans (e.g., 250–500 mg/day). Innate immune training (dectin-1 pathways).

10. Whey protein or BCAAs (20–30 g protein/day as needed). Preserves lean mass during therapy.

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Regenerative” hematologic drugs

These do not treat lymphoma directly but help recover blood counts or reduce transfusions during/after chemo or radiation, improving treatment tolerance.

1. Filgrastim (G-CSF).
   Dose: ~5 µg/kg/day SC until neutrophil recovery, per protocol.
   Function/Mechanism: Stimulates bone marrow to make neutrophils (G-CSF receptor → JAK/STAT).

2. Pegfilgrastim (long-acting G-CSF).
   Dose: 6 mg SC once per chemo cycle (timed).
   Function: Same as filgrastim with single-dose convenience.

3. Sargramostim (GM-CSF).
   Dose: ~250 µg/m²/day SC/IV per protocol.
   Function: Broad myeloid recovery (neutrophils, monocytes).

4. Epoetin alfa (ESA).
   Dose: 40,000 U SC weekly (oncology-specific indications).
   Function: Stimulates red cell production (EPO receptor); reduces transfusion needs.

5. Darbepoetin alfa (long-acting ESA).
   Dose: 2.25 µg/kg SC weekly or q2w, per label.
   Function: Longer-acting anemia support.

6. Eltrombopag (TPO receptor agonist).
   Dose: 50 mg orally daily, adjust by platelets and liver tests.
   Function: Raises platelets via c-Mpl receptor signaling.

Note: Stem cell transplantation is a procedure (not a drug) reserved for specific aggressive/relapsed lymphomas—not typical for isolated uveal MALT.

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 Procedures/Surgeries

1. Fine-needle aspiration biopsy (FNAB).
   A tiny needle samples choroid/uvea for cells to confirm lymphoma; chosen when imaging is suggestive but uncertain.

2. Trans-scleral choroidal biopsy.
   Small partial-thickness approach to obtain a core from a thicker lesion; allows architecture and immunostains.

3. Pars plana vitrectomy (diagnostic/therapeutic).
   Removes vitreous for cytology/flow and can clear dense floaters, improving vision and imaging.

4. Plaque brachytherapy placement and removal.
   Minor surgery to suture a radioactive plaque for several days; highly focal radiation for local control.

5. Enucleation (eye removal; rare).
   Last resort when eye is blind and painful or tumor uncontrollable; modern care aims to preserve the eye whenever feasible.

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Prevention & risk-reduction tips

There’s no proven way to fully prevent uveal lymphoma. These steps reduce general cancer and treatment risks and improve outcomes.

1. Regular eye exams after age 50 or if you have lymphoma history.

2. Prompt evaluation of steroid-resistant “uveitis.” Don’t assume it’s just inflammation.

3. Manage autoimmune disease to limit chronic immune activation.

4. Treat chronic infections (e.g., Hep C if present) per guidelines.

5. Avoid unnecessary long-term immunosuppression where alternatives exist.

6. Don’t smoke; seek cessation help.

7. Healthy weight, diet, and activity to support immune/metabolic health.

8. Vaccination planning (non-live as advised) before immunosuppressive therapy.

9. Eye protection and workplace safety to avoid ocular injury/inflammation triggers.

10. Keep all follow-up imaging (OCT/MRI/PET-CT) to catch progression early.

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When to see doctors urgently

• New or worsening blurred vision, floaters, flashes, or visual field loss.

• “Uveitis” that isn’t improving as expected on steroids.

• Sudden eye pain with redness or high pressure.

• B-symptoms: fevers, drenching night sweats, unexplained weight loss.

• Headache, confusion, weakness, or speech changes (possible CNS involvement).

• Any new symptom after starting treatment (fever ≥38 °C, chest pain, shortness of breath, severe rash).

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What to eat and what to avoid

1. Build plates around plants: leafy greens, colorful vegetables, legumes, whole grains, berries.

2. Choose lean proteins: fish, poultry, eggs, tofu, Greek yogurt; include omega-3-rich fish weekly.

3. Hydrate well: water first; limit sugary drinks.

4. Favor healthy fats: olive oil, nuts, seeds, avocado.

5. Small, frequent meals if appetite is low; add smoothies or soups for easy calories.

6. Limit processed meats and ultra-processed snacks.

7. Moderate alcohol (or avoid during treatment if advised).

8. Food safety focus during immunosuppression: avoid raw/undercooked meats, unpasteurized products, and buffet foods.

9. Mind salt if on steroids or with high BP; emphasize herbs/spices.

10. Coordinate supplements with your team to avoid interactions.

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Frequently asked questions

1. Is uveal lymphoma the same as uveal melanoma?
   No. Lymphoma is a cancer of immune cells; melanoma is a pigment-cell cancer. They look and behave differently.

2. Is it curable?
   Many primary uveal MALT cases are well controlled or locally “cured” with radiation. VRL/CNS-linked disease needs more intensive therapy and long-term monitoring.

3. Will I lose my eye?
   Very unlikely with modern care. Enucleation is reserved for blind, painful eyes or uncontrollable disease.

4. Can it spread to the brain?
   Vitreoretinal lymphoma has a high association with CNS disease. Primary uveal MALT has a lower risk but systemic staging is still important.

5. How is it confirmed?
   Imaging suggests it; biopsy or vitreous cytology/flow confirms cell type and guides treatment.

6. Will steroids cure it?
   No. Steroids may temporarily reduce inflammation but don’t eradicate lymphoma cells.

7. What is the usual first treatment?
   For localized uveal MALT, eye-directed radiation is common. Systemic or VRL disease may need chemo-immunotherapy and/or intravitreal therapy.

8. What side effects might I expect from eye radiation?
   Possible dry eye, cataract progression, retinal vascular changes, or mild surface irritation; risks are dose- and field-dependent and discussed during planning.

9. Do I need chemotherapy if it’s only in the eye?
   Often no for limited uveal MALT (radiation alone may suffice). Your team will stage carefully before deciding.

10. How often will I be followed?
    Typically every 3–6 months initially with eye exams and imaging; systemic surveillance as your oncologist recommends.

11. Can I keep working and driving?
    Many can, with low-vision aids and lighting adjustments; follow legal vision requirements for driving.

12. What about pregnancy?
    Treatment choices must be individualized; avoid certain drugs/radiation during pregnancy—specialist counseling is essential.

13. Should I get a second opinion?
    Yes—rare eye cancers benefit from ocular oncology center input.

14. Are clinical trials available?
    Sometimes—especially for VRL/CNS-linked disease or relapsed cases. Ask your team to search active trials.

15. Do supplements treat lymphoma?
    No. Supplements are supportive only and must be cleared by your clinicians to avoid interactions.

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 29, 2025.