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 Table of Contents  
Year : 2020  |  Volume : 32  |  Issue : 3  |  Page : 299-301

Primary conjunctival non-Hodgkin's marginal zone lymphoma

Department of Ophthalmology, Santhiram Medical College, Kurnool, Andhra Pradesh, India

Date of Submission31-Mar-2020
Date of Acceptance13-Apr-2020
Date of Web Publication23-Dec-2020

Correspondence Address:
Dr. Nareddy Prasanna
Flat No. 306, Block 4, B.K. Singh Towers, Seetharama Raju Nagar, Near K.C. Canal, Kurnool - 518 002, Andhra Pradesh
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/kjo.kjo_37_20

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A 41-year-old male patient presented with conjunctival mass in both eyes since 5 years with no history of pain or visual disturbance and nonprogressive. On examination, bilateral, pinkish, freely mobile, nontender conjunctival mass noticed on both sides of cornea, with rest of the findings being normal. No lymphadenopathy. No history of systemic illness. Surgical excision biopsy with histopathological examination of conjunctival mass of both eyes was done. Report showed low-grade Extranodal non-Hodgkin's marginal zone lymphoma. As the isolated ophthalmic features of lymphoma is as such rare, there are high chances of misdiagnosing the condition with the other ocular conditions. Suspicious presentation requires a surgical excision biopsy.

Keywords: Conjunctiva, extranodal, immunohistochemistry, salmon pink

How to cite this article:
Prasanna N. Primary conjunctival non-Hodgkin's marginal zone lymphoma. Kerala J Ophthalmol 2020;32:299-301

How to cite this URL:
Prasanna N. Primary conjunctival non-Hodgkin's marginal zone lymphoma. Kerala J Ophthalmol [serial online] 2020 [cited 2022 Dec 3];32:299-301. Available from: http://www.kjophthal.com/text.asp?2020/32/3/299/304553

  Introduction Top

Lymphoma is a malignant lymphoproliferative tumor that can involve the conjunctiva. Approximately 5%–15% of all extranodal lymphomas are found in the ocular adnexal region, with approximately 25% of those involving the conjunctiva. The most common subtype of conjunctival lymphoma is extranodal marginal zone lymphoma (EMZL) (80%).[1] Even though low-grade non-Hodgkin's marginal zone lymphoma are slow growing, and mimics other ocular conditions, it is important to diagnose the condition early to prevent the systemic spread. In our case, the mass was present since 5 years without systemic spread, which was diagnosed by excision biopsy.

  Case Report Top

A 41-year-old male patient presented with complaints of conjunctival mass in both eyes since 5 years. It was non-progressive. Negative history of pain, redness, itching, visual disturbance, trauma or any other systemic illness. On ocular examination, both eyes showed salmon pinkish mass on either side of cornea within palpebral fissure area, freely mobile, nontender with rest of the anterior segment findings being normal [Figure 1] and [Figure 2]. The posterior segment of both eyes was normal. No lymphadenopathy. Ultrasound B-scan was normal in both eyes. The patient was advised tapering dose of topical steroids, on follow-up, the condition was the same. The patient was taken for surgical excision biopsy with histopathological examination of both eyes [Figure 3]. Biopsy report showed non-Hodgkin's Lymphoma and required for immunohistochemistry for further grading. The patient was referred to systemic oncologist where tests to rule out systemic involvement were done which showed normal results. Immunohistochemistry was done, report showed positive for CD 3, CD20, CD5, Bcl 2 and negative for CD2, cyclin D1 and reported as EMZL. Patient was referred to ocular oncology center for radiation therapy, but the patient was not affordable for further treatment. Patient is been followed since 1 year and shows no signs of recurrence and yet to be followed further [Figure 4].
Figure 1: Right eye-pinkish subconjunctival mass temporally

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Figure 2: Localized subconjunctival mass, freely mobile, nontender

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Figure 3: Intraoperative excision of subconjunctival mass by delineating the surrounding structures

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Figure 4: Eye during follow-up period

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  Discussion Top

The etiology of conjunctival lymphoma remains elusive. Conjunctival lymphoma appears to develop as a result of chronic inflammation triggered by endogenous or exogenous antigens, leading to sustained proliferation that permits the potential for genetic mutations leading to eventual monoclonal B or T lymphocyte populations.[1] Although low-grade conjunctival lymphomas tends to be slow-growing, unilateral and indolent, systemic metastasis is seen in approximately 20% of patients; risk of metastasis increases in patients with a bilateral presentation.[2] While conjunctival lymphoma is primarily seen in the elderly, it has been described in individuals as young as 33 months. Chronic antigenic stimulation from pathogens including Helicobacter pylori, Chlamydia psittaci, and hepatitis C has been associated with conjunctival EMZL. Autoimmune disorders such as Sjögren syndrome, systemic lupus erythematosus, and Hashimoto thyroiditis have also been implicated in the pathogenesis of conjunctival EMZL.[2] In our case, where lymphoma is confined to the conjunctiva which is a rare presentation, there are high chances of misdiagnosing the condition with other ocular conditions such as benign lymphoid hyperplasia, nodular episcleritis, and delay in the diagnosis may lead to systemic spread, it is important to have a thorough slit lamp examination and warrants surgical excision biopsy with histopathological examination for definitive diagnosis. And detailed systemic evaluation to rule out metastasis. 5 year survival rate of lymphoma depends mainly on subtype which ranges from 97% (extranodal marginal zone Bcell lymphoma) to 9% (mantle cell lymphoma).[3] Overall, prognosis is generally good, with 90% of patients not experiencing progression or recurrence during 1 year follow-up period.[4] Low-grade lymphomas have the lowest progression, followed by higher grade diffuse large B-cell and mantle cell lymphomas.[5]

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. The patient has given his consent for his images and other clinical information to be reported in the journal.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Tanenbaum RE, Galor A, Dubovy SR, Karp CL. Classification, diagnosis, and management of conjunctival lymphoma. Eye Vis (Lond) 2019;6:22.  Back to cited text no. 1
Yumori JW, Jakirlic N, Van K. Conjunctival lymphoma: A teaching case report. Optom Educ 2019;44:3.  Back to cited text no. 2
Available from: https://eyewiki.aao.org/Conjunctival_lymphoma. [Last accessed on 2020 Jan 08].  Back to cited text no. 3
Kirkegaard MM, Coupland SE, Prause JU, Heegaard S. Malignant lymphoma of the conjunctiva. Surv Ophthalmol 2015;60:444-58.  Back to cited text no. 4
Baldini L, Blini M, Guffanti A, Fossati V, Colombi M, La Targia ML, et al. Treatment and prognosis in a series of primary extranodal lymphomas of the ocular adnexa. Ann Oncol 1998;9:779-81.  Back to cited text no. 5


  [Figure 1], [Figure 2], [Figure 3], [Figure 4]


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