Bilateral primary intraocular lymphoma in a 24-year old pregnant woman with hepatitis C: A case report

Dimitrios Zisis; Aristotelis Karamaounas; Anna Maria Sideri; Andreas Katsimpris; Iasonas Papadopoulos; Ioannis Tservakis

doi:10.4103/ijo.IJO_27_22

CASE REPORT

Year : 2022 | Volume : 2 | Issue : 3 | Page : 777-779

Bilateral primary intraocular lymphoma in a 24-year old pregnant woman with hepatitis C: A case report

Dimitrios Zisis, Aristotelis Karamaounas, Anna Maria Sideri, Andreas Katsimpris, Iasonas Papadopoulos, Ioannis Tservakis
First Department of Ophthalmology, National and Kapodistrian University of Athens, General Hospital “G. Gennimatas”, Athens, Greece

Date of Submission	04-Jan-2022
Date of Acceptance	12-Apr-2022
Date of Web Publication	16-Jul-2022

Correspondence Address:
Dr. Andreas Katsimpris
First Ophthalmology Department, “G. Gennimatas” Hospital, National and Kapodistrian University of Athens, Leof. Mesogeion 154, Athens
Greece

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijo.IJO_27_22

Abstract

Primary intraocular lymphoma (PIOL) is a rare malignancy affecting primarily elderly patients. In this case report, we present a rare case of bilateral PIOL in a 24-year-old pregnant woman with hepatitis C. The patient presented with blurred vision and floaters in the right eye for a duration of at least 3 weeks and gradual visual loss in the left eye over the course of 14 months. Fundoscopy revealed mild vitritis in the right eye and severe vitritis in the left eye. Moreover, serological testing revealed that the patient was positive for hepatitis C. Due to high clinical suspicion, a diagnostic 23-gauge pars plana vitrectomy was performed in both eyes and cytologic evaluation of the aspirate confirmed the diagnosis of PIOL. Therefore, despite PIOL being of low prevalence, chronic persistent vitritis in young adults should raise suspicion about this malignancy.

Keywords: Hepatitis C, primary central nervous system lymphoma, primary intraocular lymphoma

How to cite this article:
Zisis D, Karamaounas A, Sideri AM, Katsimpris A, Papadopoulos I, Tservakis I. Bilateral primary intraocular lymphoma in a 24-year old pregnant woman with hepatitis C: A case report. Indian J Ophthalmol Case Rep 2022;2:777-9

How to cite this URL:
Zisis D, Karamaounas A, Sideri AM, Katsimpris A, Papadopoulos I, Tservakis I. Bilateral primary intraocular lymphoma in a 24-year old pregnant woman with hepatitis C: A case report. Indian J Ophthalmol Case Rep [serial online] 2022 [cited 2022 Aug 11];2:777-9. Available from: https://www.ijoreports.in/text.asp?2022/2/3/777/351146

Lymphomas comprise a heterogeneous group of disorders, resulting from the clonal proliferation of lymphocytes, which are broadly classified into two categories, namely, Hodgkin and non-Hodgkin lymphomas (NHL).^[1] Although the majority of NHLs arise from B-lymphocytes,^[2] non-Hodgkin lymphomas originate from T-lymphocytes, natural killer cells and their precursors.^[3] Primary central nervous system lymphoma (PCNSL) is an uncommon extra-nodal non-Hodgkin lymphoma, comprising around 3% of all CNS tumors and usually involves the brain, spinal cord, or eyes. Primary intraocular lymphoma (PIOL), a subset of PCNSL, affects the retina and less commonly the uvea^[4] and occurs usually in elderly patients. The incidence of PIOL in young adults is low and thus, we report this rare case of a 24-year-old patient with bilateral PIOL, who was simultaneously positive for hepatitis C.

Case Report

The patient was a 24-year-old pregnant woman of Egyptian descent who presented to our tertiary referral center. She complained of blurred vision and floaters in her right eye for a duration of at least 3 weeks and gradual loss of vision in her left eye over the course of 14 months, the latter having originally presented as blurred vision and floaters.

Her past medical history was unknown and she denied having any systemic disease.

The patient had been conservatively treated with 0.1% dexamethasone eye drops for 3 weeks without any apparent improvement in symptoms and was thus referred to our vitreoretinal department for further investigation.

Upon presentation, visual acuity was 8/10 in the right eye and CF in the left eye, whereas the IOP was 11 and 13 mmHg, respectively. In both eyes, slit-lamp biomicroscopy showed normal corneas, Tyndall (+), no anterior or posterior synechiae, and clear lenses. Moreover, it revealed mild and severe vitritis in the right and left eyes, respectively. Fundoscopy revealed no pathology in the right eye but a persistent vitreous hemorrhage in the left eye. In addition, our systemic workup included complete immunological laboratory tests, which revealed the presence of hepatitis C. The patient was also examined by physicians specialized in uveitis and due to high clinical suspicion of intraocular lymphoma, a diagnostic 23-gauge pars plana vitrectomy was performed in the left eye of the patient. Fundus photography, optical coherence tomography (OCT), and fundus autofluorescence of both eyes were performed after vitrectomy of the left eye [shown in [Figure 1], [Figure 2], [Figure 3]]. Cytologic evaluation and flow cytometry of the aspirate confirmed the diagnosis. The patient's young age has also led to a 23-gauge pars plana vitrectomy of the right eye, as well, to confirm the diagnosis. Flow cytometry revealed CD45 + cells, IgM cells with kappa monoclonal light chains, CD79b+, and the presence of mitotic index CD71. The patient was referred to the oncology department but refused further investigation and treatment.

Figure 1: Preoperative (a) and postoperative (b, after pars plana vitrectomy) color fundus images of the right (a) and left (b) eyes, respectively. The fundus image of the right eye (a) is blurry due to vitreous hazing

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Figure 2: Postoperative OCT (a, after pars plana vitrectomy) of the left eye showing disruption of the inner and outer segment junctions without cystoid macular edema and preoperative OCT (b) of the right eye with vitreous hazing. * * OCT: optical coherence tomography

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Figure 3: Preoperative (a) and postoperative (b, after pars plana vitrectomy) fundus autofluorescence of the right (a) and left (b) eyes, respectively

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Discussion

Despite the high prevalence of PCNSL in immunocompromised patients (e.g., patients with HIV infection and organ recipients), the majority of individuals with PCNSL are immunocompetent. PIOL, a common subset of PCNSL, presents usually as an aggressive lymphoma, originating usually from B-cells and their precursors and affecting vitreoretinal tissues. Over 15% of patients with PCNSL will eventually develop PIOL and conversely, 65% to 90% of patients with PIOL will develop PCNSL.^[5] Age-of-onset can vary in PIOL from 15 to 85 years, with a mean age that ranges from the late 50s to the early 60s.^[6]

Patients with PIOL present usually with decreased visual acuity and floaters caused by vitritis and less commonly with ocular pain, exudative retinal detachment, or glaucoma.^[7] In cases where there is no CNS involvement, PIOL can be erroneously diagnosed as chronic intermediate uveitis, which is steroid-resistant, and clinical examination may reveal cells in the vitreous and/or vitreous haze. Additionally, multiple orange-yellow subretinal lesions (leopard spot pattern) constitute a pathognomonic, albeit uncommon, retinal finding in patients with PIOL.^[7]

Currently, the median survival time for patients with PIOL ranges from 1 to over 6 years, depending mainly on whether the CNS is involved or not and the type of therapy used.^[8],[9] Despite the masquerading of PIOL, ocular examination in conjunction with ophthalmic imaging and laboratory tests can lead to its diagnosis. Common diagnostic techniques for PIOL include OCT, ultrasonography, magnetic resonance imaging, and cytological studies. Vitreous sampling remains the most important examination, as flow cytometry of the aspirated vitreous is the gold standard examination and can determine the monoclonality of lymphocytes.^[4] The interleukin-10 to interleukin-6 ratio can help distinguish between intraocular lymphoma and chronic inflammation, with the ratio being greater than 1 and lower than 1, respectively. Lastly, MYD88 mutation analysis can hint toward the diagnosis of PIOL.

In our case, arriving at the diagnosis of PIOL posed a challenge, firstly because we assumed that the vitreous inflammation was caused by the vitreous hemorrhage and secondly due to the lack of systemic symptoms. When the diagnosis was made, we referred the patient to the oncology department for further investigation and treatment. Magnetic resonance imaging scan, termination of pregnancy, and chemotherapy were proposed to the patient, but she refused further imaging and treatment. The presence of hepatitis C in our patient could be an incidental finding or it could be the cause of some ocular findings, considering the fact that hepatitis C has been associated with dry eye syndrome, retinal vasculitis, and intermediate uveitis.^[10] Several epidemiological studies have shown an increased risk of B cell non-Hodgkin lymphomas in HCV-infected patients with a wide geographic variability although a correlation between HCV infection and PIOL has yet to be found.

The diagnosis and treatment of PCNSL and PIOL call for a multidisciplinary approach, ideally in large centers under the guidance of experienced specialists. Despite PCNSL being sensitive to chemotherapy and radiotherapy, relapse-free survival remains poor, when compared to other lymphomas, as most therapies available for systemic lymphoma have been proven ineffective for PCNSL.^[4] Currently, there is no consensus on the treatment of PIOL, which currently entails a combination of radiotherapy, systemic chemotherapy, and local ocular treatment. Pregnancy further complicates appropriate treatment, considering the fact that specific modalities such as systemic chemotherapy pose a threat to the developing fetus. However, external beam radiation therapy and intravitreal chemotherapy without systemic chemotherapy have been found to be safe and effective approaches in gravid patients with PIOL, who do not wish to terminate their pregnancy.^[11] Unfortunately, these treatment options were not available in our hospital and therefore could not be offered to our patient. Whether ocular radiotherapy or intravitreal chemotherapy is the appropriate first-line treatment and whether they need to be combined with systemic chemotherapy or not, needs to be determined by future studies. Last but not least, due to high relapse rates, patients should be monitored closely with regular magnetic resonance imaging, and neurological and ocular examinations.

Conclusion

To the best of our knowledge, this is the youngest patient with bilateral PIOL that has been described in the literature. PIOL is a rare malignancy that lacks a concrete therapeutic approach and is characterized by a constellation of ocular manifestations that can render its diagnosis challenging. In conclusion, we deem it important that PIOL be suspected in young adults presenting with chronic persistent vitritis.

Declaration of patient consent

Verbal and written informed consent was obtained from the patient for publication of this case report and all accompanying images.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

Authors' contributions

All authors collected and interpreted the patient's clinical findings. A. K., D.Z., and A. M. S. contributed to the writing and editing of the manuscript. I.T. reviewed the literature. All authors read and approved the final version of the manuscript.

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