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Orbital lymphoma - External and Internal Eye
Other Resources UpToDate PubMed

Orbital lymphoma - External and Internal Eye

Contributors: Aditi Jani MD
Other Resources UpToDate PubMed

Synopsis

Orbital lymphoma is a type of non-Hodgkin lymphoma that can originate from the extraocular muscles, the lacrimal gland, the conjunctiva, or the eyelids. The majority of orbital lymphomas are B-cell type or mucosa-associated lymphoid tissue (MALT) type and can be primary in nature or secondary, associated with metastatic disease. Other subtypes that have been reported include follicular lymphoma and mantle cell lymphoma.

Orbital lymphoma makes up 55% of all orbital malignancies and has been reported to make up between 1% and 10% of all non-Hodgkin lymphomas. It can present unilaterally or bilaterally and typically affects older adult patients between their fifth and seventh decades. It is most commonly reported in Asia and Europe.

Symptoms include double vision, eyelid swelling, proptosis, difficulty with eye movement, redness, and rarely pain.

The most reported risk factor is immunosuppression such as AIDS, status post-organ transplant, and increasing age. Recent associations with infections, autoimmune conditions, and genetic factors have been reported in varying degrees. The associated infections include Chlamydia species, Helicobacter pylori, and Epstein-Barr virus. The associated autoimmune conditions include rheumatoid arthritis, Graves disease, and Sjögren syndrome.

Codes

ICD10CM:
C69.60 – Malignant neoplasm of unspecified orbit

SNOMEDCT:
13048006 – Orbital lymphoma

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Last Reviewed:12/31/2019
Last Updated:05/06/2020
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Orbital lymphoma - External and Internal Eye
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Orbital lymphoma : Diplopia, Proptosis
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