Case Presentation: 60 year old African American man with history of HTN, HLD, and mitral valve regurgitation presented to the ER complaining of 2.5 weeks of progressively worsening right eye pain and swelling. [shmabstracts.com]

One year later he presented with an acute confusional episode that resolved over a two week period. [jneuro.com]

[…] of the disease often discloses little-to no atypicality in the lymphocytes present in the specimen. [mersi.com]

When lymphomas are present on the eye and nowhere else, they are treated with EBRT-external beam radiation therapy. [verywellhealth.com]

Nine patients (47%) presented with proptosis and five patients (26%) presented with an upper lid mass; three (15%) as conjunctival mass and two (11%) as lacrimal gland swelling [Table - 2]. [ijo.in]

• Lymphadenopathy

  Doctors use the term localized lymphadenopathy to refer to swelling in a single lymph node or a small cluster of lymph nodes, such as the PLNs. Localized lymphadenopathy happens when there is a problem in nearby tissues. [medicalnewstoday.com]

   The lacrimal gland, lacrimal sac, EOM can be similarly involved. 10. 06/14/15 Systemic disease to be suspected Cervical lymphadenopathy, Preauricular lymphadenopathy Parotid gland swelling Abdominal mass Thorough physical evaluation 11. [slideshare.net]

  The CT scan showed small volume mediastinal lymphadenopathy. The blood count and bone marrow studies were normal and there was no monoclonal immunoglobulin. The CSF was normal. [jneuro.com]

  Enlarged, matted, and non-tender cervical lymphadenopathy was also found. There was marked restriction of the right eye on adduction. The rest of the anterior and posterior segment examinations were unremarkable in both eyes. [djo.harvard.edu]

  The patient denied any known history of a recent viral illness, and preauricular, submandibular and cervical lymphadenopathy were absent. The patient was referred to a corneal specialist for a conjunctival biopsy. [journal.opted.org]

• Aspiration

  The reasons why cytology of the vitreous aspirate does not always reveal malignant cells are numerous. The aspirate only contains a small number of cells. [bjo.bmj.com]

  The diagnosis of intra- ocular lymphoma was made from a subretinal aspiration showing a population of monoclonal Blymphoplasmacytoid lymphocytes. [jneuro.com]

  Before instituting infusion, however one cc of “neat,” undiluted vitreous was aspirated into a three cc syringe with an attached 20 gauge needle. [mersi.com]

  The standard approach to diagnosis of PVRL is vitrectomy or vitreous aspirate biopsy. [onlinelibrary.wiley.com]

  Correct diagnosis thus depends on a high index of suspicion and frequently requires radiologic imaging, histologic analysis, particularly vitreous biopsy or flow cytometry, subretinal aspiration and retinal biopsy. [annalsafrmed.org]

• Abdominal Mass

  mass Thorough physical evaluation 11. [slideshare.net]

• Sweating

  […] at Mayo Clinic Types Symptoms Signs and symptoms of non-Hodgkin's lymphoma may include: Painless, swollen lymph nodes in your neck, armpits or groin Abdominal pain or swelling Chest pain, coughing or trouble breathing Persistent fatigue Fever Night sweats [mayoclinic.org]

  […] unexplained weight loss pain in the abdomen indigestion and bloating nausea and vomiting abnormal pressure in the affected area congestion in the face, neck, and upper chest constant coughing flu-like body aches bone pain a recurrent or persistent fever night sweats [medicalnewstoday.com]

  In March, he felt middle and upper abdominal pain, with abdominal distension, nausea, fatigue and profuse sweating. A few days later, he began to feel blurred vision OD, with water ripple feeling. [bmcophthalmol.biomedcentral.com]

• Blurred Vision

  Common symptoms of ocular lymphoma include: • Blurred vision • Vision loss • Seeing floaters • Eye redness/swelling • Sensitivity to light Treatment options include: • Chemotherapy: the use of special drugs to kill cancer cells both inside the eye and [myeyeassociates.com]

  In Dec 2006, she complained of blurred vision and red eye OU, tinnitus and hearing loss of the right ear, as well as rhinobyon. [bmcophthalmol.biomedcentral.com]

  Eye Lymphoma Symptoms The most common symptoms for eye lymphoma include blurred vision, decrease or loss of vision, and floaters in vision. Redness or swelling in the eye and sensitivity to light may also occur. Eye pain is uncommon. [sflhealthandwellness.com]

  Symptoms of eye cancer include bulging of the eye, some loss of sight, blurred vision, dark spot on the iris, flashing lights, shadows and misting of the eye lens, and eye pain. [cancer.ie]

  Symptoms of eye cancer can include: shadows, flashes of light, or wiggly lines in your vision blurred vision a dark patch in your eye that's getting bigger partial or total loss of vision bulging of 1 eye a lump on your eyelid or in your eye that's increasing [nhs.uk]

• Dry Eyes

  An eye doctor will follow a patient undergoing ocular radiation closely because of known side effects such as severe dry eye, cataract development, ischemic optic neuropathy, optic atrophy, and neovascular glaucoma. [verywellhealth.com]

  Adequate management of the patient's dry eyes, cataracts, and laser photocoagulation of radiation retinopathy is important. [nature.com]

  Treatment External beam radiation is the primary treatment for MALT lymphoma. 22 The main side effects of radiotherapy include dry eye, cataract development, reduced visual acuity and radiation chorioretinopathy. [reviewofoptometry.com]

  Orbital radiation can lead to prolonged remission, but the adverse effects are many: radiation retinopathy, optic neuropathy, and dry eye; furthermore, radiation does not necessarily prolong survival.29 Rituximab, a monoclonal antibody against the CD20 [archivesofpathology.org]

  Two patients who received chemotherapy but no EBRT developed ocular relapse at a follow-up time of 14 and 31 months. 30 Radiation-related side effects included optic neuropathy, retinopathy, conjunctivitis, dry eyes, cataracts and glaucoma. 31 In most [reviewofophthalmology.com]

• Anterior Uveitis

  In our case, both non-granulomatous anterior uveitis and vitritis with macular edema were detected as the first clinical signs of HL. [ncbi.nlm.nih.gov]

  The diagnosis was “granulomatous uveitis OU”. Anterior uveitis did not respond well to topical 1% Pred Forte, so systemic corticosteroid and azathioprine were given. [bmcophthalmol.biomedcentral.com]

  Immunophenotypic characterization of an unusual T-cell lymphoma presenting as anterior uveitis. A clinicopathological case report. Arch Ophthalmol 1989; 107 : 1349–1353. 20 Davis JL, Viciana AL, Ruiz P. [nature.com]

  […] scleritis. [26] The anterior scleritis may be associated with uveal effusion syndrome. [26] Lymphoma may masquerade as iritis, anterior uveitis or panuveitis. [2], [3], [7], [12], [25], [26], [27] Incorrect diagnosis of the uveitic syndrome may have [annalsafrmed.org]

• Conjunctival Hyperemia

  hyperemia 10 (17) 5 (14) 0 0 Ptosis 9 (15) 1 (3) 2 (50) 2 (50) Symptoms Tumor 15 (25) 25 (68) 3 (75) 3 (75) Proptosis 26 (43) 0 1 (25) 0 Red eye 12 (20) 12 (32) 0 0 Pain 12 (20) 2 (5) 0 1 (25) B-symptoms 5 (8) 0 0 1 (25) Symptom duration in months, median [iovs.arvojournals.org]

  hyperemia and transient keratopathy. 24 Early evidence suggests that rituximab may have fewer side effects and require fewer injections to achieve remission, however further investigation on larger numbers of patients is required. 23 Large retrospective [reviewofophthalmology.com]

• Prolapse

  Conjunctival masses may mimic amelanotic melanoma, age-related sub-conjunctival fat prolapse and certain other rare conjunctival tumors such as myxoma, juvenile xanthogranuloma and leiomyosarcoma. [omicsonline.org]

DIAGNOSIS PCNSL should be suspected in any patient with posterior uveitis who has a negative workup for common causes of posterior uveitis. [retinatoday.com]

Clinical awareness of any atypical ocular presentations in NKTCL cases should necessitate thorough workup in order not to hinder an early discovery of disease relapse. References Armitage JO: The aggressive peripheral T-cell lymphomas. [cureus.com]

CSF dissemination is detected during staging workup in only 10–15% of cases (Grimm et al, 2007 ). [onlinelibrary.wiley.com]

Hence, all patients with ocular lymphoma should have a complete workup to rule out systemic lymphoma. " ________________________ Intra-ocular lymphoma: A rare malignant form of eye cancer. [lymphomation.org]

• Helicobacter Pylori

  The association between conjunctival MALT lymphoma and Helicobacter pylori. Br J Ophthalmol. 2008 Apr;92(4):534–6. Ferreri AJM, Govi S, Pasini E, Mappa S, Bertoni F, Zaja F, et al. [revistafarmaciahospitalaria.sefh.es]

  Bacteria linked to an increased risk of non-Hodgkin's lymphoma include the ulcer-causing Helicobacter pylori. Chemicals. Certain chemicals, such as those used to kill insects and weeds, may increase your risk of developing non-Hodgkin's lymphoma. [mayoclinic.org]

  Etiopathogenesis and Immunology Lymphoma in gastro-intestinal tract is observed to be associated with Helicobacter pylori in more than 90% cases [ 24 ]. [omicsonline.org]

  Breath test followed by gastric biopsy to test Helicobacter pylori status is also indicated (Sassone et al., 2017). Cp infection has to be assessed in peripheral blood and conjunctival swabs by PCR. [atlasgeneticsoncology.org]

  Chronic antigenic stimulation from pathogens including Helicobacter pylori, Chlamydia psittaci and hepatitis C 2 has been associated with conjunctival EMLZ. [journal.opted.org]

The following are treatment options for lymphoma of the eye. Your healthcare team will suggest treatments based on your needs and work with you to develop a treatment plan. [cancer.ca]

He continues, "I hope that this treatment protocol would become the impetus for breaking down the poor prognosis of this disease." [u-tokyo.ac.jp]

Recurring tumors have worse prognosis compared to tumors that do not recur Response to treatment: Tumors that respond to treatment have better prognosis compared to tumors that do not respond to treatment Progression of the condition makes the outcome [dovemed.com]

The authors found that doxycycline treatment caused lymphoma to regress in both patients who did not test positive for the disease and those who did. [sciencedaily.com]

Among eye diseases, intraocular lymphoma has the worst survival prognosis. [u-tokyo.ac.jp]

Prognosis is less favourable. [radiopaedia.org]

Many people are diagnosed with eye lymphoma after it has already spread to the brain, which has a worse prognosis. It is important to remember that statistics on the survival rates for people with eye cancer are an estimate. [cancer.net]

The patient was admitted to our clinic for etiologic study and treatment. [ncbi.nlm.nih.gov]

Pathogenesis Etiology The eye itself is not the site of the lymphoma. There may be enlargement on the bulbar surface of the glans nictitans. One or both of the eyes may be affected. [vetstream.com]

The possible infective etiology may explain the probable increase in the incidence of orbital lymphomas (see below) 3. Typically patients are between 50 and 70 years of age, with no recognized gender predilection 6. [radiopaedia.org]

The differential diagnosis includes infectious, inflammatory, and other neoplastic processes.22 Infectious etiologies include endophthalmitis secondary to bacterial, viral, fungal, or parasitic infections.22 A vitreous sample is obtained and should be [archivesofpathology.org]

More rarely, an infectious etiology should also be considered. [djo.harvard.edu]

Epidemiology Neoplasia of the eye and orbit is uncommon in ferrets. Malignant lymphoma is the most common neoplasm in both the eye and the orbit. [vetstream.com]

"Epidemiology of primary CNS lymphoma". Journal of Neuro-Oncology. 43 (3): 199–201. doi : 10.1023/A:1006290032052. PMID 10563423. ^ Ahmed, S; Shahid, RK; Sison, CP; Fuchs, A; Mehrotra, B (February 2006). [en.wikipedia.org]

It provides a thorough review of conjunctival anatomy and physiology along with explanations of key findings, common clinical presentations, differential diagnoses, epidemiology, pathophysiology, risk factors, treatment and management options and prognosis [journal.opted.org]

PMID 28682481 Descriptive epidemiology of ophthalmic and ocular adnexal non-Hodgkin's lymphoma. Moslehi R, Coles FB, Schymura MJ Expert Rev Ophthalmol. 2011;6:175-180. [atlasgeneticsoncology.org]

Fisher SG, Fisher RI (2004) The epidemiology of non-Hodgkin's lymphoma. Oncogene 23: 6524-6534. Palackdharry CS (1994) The epidemiology of non-Hodgkin's lymphoma: Why the increased incidence? Oncology (Williston Park) 8: 67-73. [omicsonline.org]

A review of ocular lymphoma diagnosis, pathophysiology, and management is undertaken. Format: video Series: Moran Eye Center Grand Rounds Identifier: Moran_CORE_15769 Copyright statement: Copyright 2015. [morancore.utah.edu]

It provides a thorough review of conjunctival anatomy and physiology along with explanations of key findings, common clinical presentations, differential diagnoses, epidemiology, pathophysiology, risk factors, treatment and management options and prognosis [journal.opted.org]

A pathophysiologic scenario involving chronic and sustained stimulation of the immune system leading to lymphoid transformation has emerged. [scienceopen.com]

Pathophysiology [ edit ] Recent studies [ by whom? ] have detected the presence of viral DNA in ocular lymphoma cells. This implies that pathogens play a role in ocular lymphoma. [en.wikipedia.org]

Pathophysiology of retinal lymphoma. Ocul Immunol Inflamm. 2009;17(4):227–237. 6. Char DH, Ljung BM, Deschênes J, Miller TR. Intraocular lymphoma: immunological and cytological analysis. Br J Ophthalmol. 1988;72(12):905–911. 7. [dovepress.com]

This can help prevent cancer from spreading there or help destroy hidden cancer cells. [columbiaeye.org]

This finding shows that aggressive systemic chemotherapy and preventive radiotherapy could greatly improve the prognosis of the disease. "Intraocular malignant lymphoma, although a disease of the eye, is intractable and life-threatening. [u-tokyo.ac.jp]

Sometimes patients take a special antibiotic to prevent long-term involvement elsewhere in the body. Conjunctival Lymphoma Photos [willseye.org]

[…] at risk for getting eye lymphoma include those who: have rheumatoid arthritis or other autoimmune diseases have certain medical problems that affect their immune system have AIDS (acquired immunodeficiency syndrome) are taking certain medications to prevent [aao.org]

Radiation therapy may also be directed to the brain and spinal cord to help prevent the cancer from spreading there or to help destroy undetected cancer cells that could be there. Lauren R. Rosecan M.D., Ph.D., F.A.C.S. [sflhealthandwellness.com]