Intraocular lymphoma

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Intraocular lymphoma is a rare malignant form of eye cancer. Intraocular lymphoma may affect the eye secondarily from a metastasis from a non-ocular tumor or may arise within the eye primarily (primary intraocular lymphoma, PIOL). PIOL is a subset of primary central nervous system lymphoma (PCNSL). PCNSL (and PIOL) are most commonly a diffuse large B-cell immunohistologic subtype of non-Hodgkin's lymphoma according to the World Health Organization (WHO) classification of lymphomas. The most common symptoms of PIOL include blurred or decreased vision due to tumor cells in the vitreous. Most cases of PIOL eventuate to central nervous system involvement (PCNSL) while only 20% of PCNSL lead to intraocular (PIOL) involvement. PIOL and PCNSL remain enigmas because both structures are immunologically privileged sites (the brain sits behind the blood–brain barrier and the retina sits behind the blood-retinal barrier) and so do not normally have immune cells trafficking through these structures. What is more, while the vast majority of PCNSL in patients with acquired immune deficiency syndrome (AIDS) is related to the Epstein-Barr virus (EBV), the development of PCNSL and PIOL in immunocompetent patients is unknown and shows no general relation to infectious DNAs. [1]

PIOL affects the sub-retinal pigment epithelium (RPE), can invade into the retina, the vitreous, and the optic nerve. Ophthalmoscopy frequently reveals creamy yellow-to-orange colored subretinal infiltrates. Fluorescein angiography may reveal "leopard spot" patterns due to sub-RPE infiltrates that stain early and progressively or mottling of the RPE due to hyper- and hypofluorescent window defects. [2]

PIOL is known as a masquerade syndrome because it frequently simulates the signs and symptoms of uveitis. As such, PIOL is frequently treated with corticosteroids. Occasionally, PIOL has mimicked a retinitis and has been treated with antiviral medication. It is not until the supposed uveitis fails to respond to treatment, becomes recalcitrant to treatment, or shows worsening with discontinuation of corticosteroid treatment that another cause is sought out. If PIOL is suspected, it is important to first obtain a magnetic resonance image (MRI) of the brain to rule out cerebral involvement (PCNSL). If MRI is negative, lumbar puncture with cerebrospinal fluid (CSF) cytology should be performed to further rule out CNS disease. Histopathologic identification of atypical lymphocytes is considered the gold standard for diagnosing PCNSL/PIOL. If CSF cytology is negative or inconclusive and PIOL is suspected, a vitrectomy is often performed with cytologic analysis. Furthermore, adjunctive testing including polymerase chain reaction (PCR) amplification to identify monoclonal rearrangements of the immunoglobulin heavy chain (IgH) gene (for B-cell lymphomas) or T-cell receptor (TCR, for the very rare T-cell lymphomas) can be performed. [3] [4] [5] Analysis of vitreous humor or aqueous humor for the P265P mutation of MYD88 is especially helpful as that mutation is found in about 80-90% of cases. [6]

Previously, radiation therapy was the mainstay treatment for PCNSL/PIOL, but methotrexate has now become first-line. [7]

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The National Eye Institute (NEI) is part of the U.S. National Institutes of Health (NIH), an agency of the U.S. Department of Health and Human Services. The mission of NEI is "to eliminate vision loss and improve quality of life through vision research." NEI consists of two major branches for research: an extramural branch that funds studies outside NIH and an intramural branch that funds research on the NIH campus in Bethesda, Maryland. Most of the NEI budget funds extramural research.

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<span class="mw-page-title-main">Uveitis</span> Inflammation of the uvea of the eye

Uveitis is inflammation of the uvea, the pigmented layer of the eye between the inner retina and the outer fibrous layer composed of the sclera and cornea. The uvea consists of the middle layer of pigmented vascular structures of the eye and includes the iris, ciliary body, and choroid. Uveitis is described anatomically, by the part of the eye affected, as anterior, intermediate or posterior, or panuveitic if all parts are involved. Anterior uveitis (iridocyclitis) is the most common, with the incidence of uveitis overall affecting approximately 1:4500, most commonly those between the ages of 20–60. Symptoms include eye pain, eye redness, floaters and blurred vision, and ophthalmic examination may show dilated ciliary blood vessels and the presence of cells in the anterior chamber. Uveitis may arise spontaneously, have a genetic component, or be associated with an autoimmune disease or infection. While the eye is a relatively protected environment, its immune mechanisms may be overcome resulting in inflammation and tissue destruction associated with T-cell activation.

<span class="mw-page-title-main">Birdshot chorioretinopathy</span> Medical condition

Birdshot chorioretinopathy, now commonly named birdshot uveitis or HLA-A29 uveitis, is a rare form of bilateral posterior uveitis affecting both eyes. It causes severe, progressive inflammation of both the choroid and retina.

<span class="mw-page-title-main">Intravitreal administration</span>

Intravitreal administration is a route of administration of a drug, or other substance, in which the substance is delivered into the vitreous humor of the eye. "Intravitreal" literally means "inside an eye". Intravitreal injections were first introduced in 1911 when Ohm gave an injection of air into the vitreous humor to repair a detached retina. In the mid-1940s, intravitreal injections became a standard way to administer drugs to treat endophthalmitis and cytomegalovirus retinitis.

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<span class="mw-page-title-main">Intermediate uveitis</span> Medical condition

Intermediate uveitis is a form of uveitis localized to the vitreous and peripheral retina. Primary sites of inflammation include the vitreous of which other such entities as pars planitis, posterior cyclitis, and hyalitis are encompassed. Intermediate uveitis may either be an isolated eye disease or associated with the development of a systemic disease such as multiple sclerosis or sarcoidosis. As such, intermediate uveitis may be the first expression of a systemic condition. Infectious causes of intermediate uveitis include Epstein–Barr virus infection, Lyme disease, HTLV-1 virus infection, cat scratch disease, and hepatitis C.

<span class="mw-page-title-main">Intraocular hemorrhage</span> Medical condition

Intraocular hemorrhage is bleeding inside the eye. Bleeding can occur from any structure of the eye where there is vasculature or blood flow, including the anterior chamber, vitreous cavity, retina, choroid, suprachoroidal space, or optic disc.

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<span class="mw-page-title-main">Orbital lymphoma</span> Human disease of the eye

Orbital lymphoma is a common type of non-Hodgkin lymphoma that occurs near or on the eye. Common symptoms include decreased vision and uveitis. Orbital lymphoma can be diagnosed via a biopsy of the eye and is usually treated with radiotherapy or in combination with chemotherapy.

<span class="mw-page-title-main">Serpiginous choroiditis</span> Medical condition

Serpiginous choroiditis, also known as geographic helicoid peripapillary choroidopathy (GHPC), is a rare, chronic, progressive, and recurrent bilateral inflammatory disease involving the retinal pigment epithelium (RPE), the choriocapillaries, and the choroid. It affects adult men and women equally in the second to seventh decades of life.

<span class="mw-page-title-main">Vogt–Koyanagi–Harada disease</span> Medical condition

Vogt–Koyanagi–Harada disease (VKH) is a multisystem disease of presumed autoimmune cause that affects melanin-pigmented tissues. The most significant manifestation is bilateral, diffuse uveitis, which affects the eyes. VKH may variably also involve the inner ear, with effects on hearing, the skin, and the meninges of the central nervous system.

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<span class="mw-page-title-main">Secondary glaucoma</span> Eye disorder

Secondary glaucoma is a collection of progressive optic nerve disorders associated with a rise in intraocular pressure (IOP) which results in the loss of vision. In clinical settings, it is defined as the occurrence of IOP above 21 mmHg requiring the prescription of IOP-managing drugs. It can be broadly divided into two subtypes: secondary open-angle glaucoma and secondary angle-closure glaucoma, depending on the closure of the angle between the cornea and the iris. Principal causes of secondary glaucoma include optic nerve trauma or damage, eye disease, surgery, neovascularization, tumours and use of steroid and sulfa drugs. Risk factors for secondary glaucoma include uveitis, cataract surgery and also intraocular tumours. Common treatments are designed according to the type and the underlying causative condition, in addition to the consequent rise in IOP. These include drug therapy, the use of miotics, surgery or laser therapy.

<span class="mw-page-title-main">Uveitic glaucoma</span> Glaucoma caused by uveitis or its treatments

Uveitic glaucoma is most commonly a progression stage of noninfectious anterior uveitis or iritis.

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<span class="mw-page-title-main">Vitritis</span> Medical condition

Vitritis is an inflammation of the vitreous characterized by vitreous white blood cells. It is a form of uveitis and is associated with anterior uveitis and more commonly intermediate or posterior uveitis. It is important to distinguish vitritis from other types of cells in the vitreous cavity such as red blood cells, pigment cells, and tumor cells. The underlying etiology of the inflammation must be determined so that appropriate treatment can be given.

References

  1. ↑ Shen, DF; Herbort, CP; Tuaillon, N; Buggage, RR; Egwuagu, CE; Chan, CC (Oct 2001). "Detection of Toxoplasma gondii DNA in primary intraocular B-cell lymphoma". Modern Pathology. 14 (10): 995–9. doi: 10.1038/modpathol.3880424 . PMID   11598169.
  2. ↑ Cassoux, N; Merle-Beral, H; Leblond, V; Bodaghi, B; Miléa, D; Gerber, S; Fardeau, C; Reux, I; Xuan, KH; Chan, CC; LeHoang, P (Dec 2000). "Ocular and central nervous system lymphoma: clinical features and diagnosis". Ocular Immunology and Inflammation. 8 (4): 243–50. doi:10.1076/ocii.8.4.243.6463. PMID   11262654. S2CID   39345449.
  3. ↑ Shen, DF; Zhuang, Z; LeHoang, P; Böni, R; Zheng, S; Nussenblatt, RB; Chan, CC (Sep 1998). "Utility of microdissection and polymerase chain reaction for the detection of immunoglobulin gene rearrangement and translocation in primary intraocular lymphoma". Ophthalmology. 105 (9): 1664–9. doi: 10.1016/S0161-6420(98)99036-4 . PMID   9754175.
  4. ↑ Coupland, Sarah E.; Anastassiou, Gerasimos; Bornfeld, Norbert; Hummel, Michael; Stein, Harald (March 2005). "Primary intraocular lymphoma of T-cell type: report of a case and review of the literature". Graefe's Archive for Clinical and Experimental Ophthalmology. 243 (3): 189–197. doi:10.1007/s00417-004-0890-2. PMID   15806372. S2CID   24772799.
  5. ↑ Gonzales, John A.; Chan, Chi-Chao (August 2007). "Biopsy techniques and yields in diagnosing primary intraocular lymphoma". International Ophthalmology. 27 (4): 241–250. doi:10.1007/s10792-007-9065-6. PMC   2048742 . PMID   17440686.
  6. ↑ Ferreri AJM et al. (2021) MYD88 L265P mutation and interleukin-10 detection in cerbrospinal fluid are highly specific discriminating markers in patients with primary central nervous system lymphoma: results from a prospective study. Br. J. Haematol. 193:497-505. doi= 10.1111/bjh.17357
  7. ↑ Anthony, Casey L.; Bavinger, J. Clay; Shantha, Jessica G.; O’Keefe, Ghazala D.; Pearce, William A.; Voloschin, Alfredo; Grossniklaus, Hans E.; Yeh, Steven (2021-12-04). "Clinical outcomes following intravitreal methotrexate for primary vitreoretinal lymphoma". International Journal of Retina and Vitreous. 7 (1): 72. doi: 10.1186/s40942-021-00346-0 . ISSN   2056-9920. PMC   8645085 . PMID   34863313.
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