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 Table of Contents  
Year : 2022  |  Volume : 2  |  Issue : 2  |  Page : 494-496

Masquerade versus masquerade: An unusual presentation of systemic B-cell lymphoma misdiagnosed as giant cell arteritis - A case report

Department of Ophthalmology, University Hospital Southampton, Tremona Road, Southampton- SO16 6YD, United Kingdom

Date of Submission08-Nov-2021
Date of Acceptance08-Dec-2021
Date of Web Publication13-Apr-2022

Correspondence Address:
Sajjad Abbas
University Hospital Southampton, Tremona Road, Southampton- SO16 6YD
United Kingdom
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijo.IJO_2808_21

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This is an atypical uniocular presentation of systemic diffuse large B-cell lymphoma. An 80-year-old Caucasian female presented with acute left visual loss, anorexia, headache, and jaw pain with disk swelling. Systemic steroids for giant cell arteritis were started despite negative temporal artery biopsy. Later, an inferior exudative retinal detachment developed. Lytic skull lesions were eventually seen on magnetic resonance imaging. Diffuse large B-cell lymphoma was diagnosed postmortem. This is the second documented case of unilateral serous retinal detachment with no uveitis/pseudouveitis or choroidal infiltrate due to lymphoma. It demonstrates pitfalls of pattern recognition when pieces of the jigsaw do not fit.

Keywords: Exudative Retinal Detachment, giant cell arteritis, lymphoma, masquerade, temporal arteritis

How to cite this article:
Abbas S, Lewis K, Rennie C. Masquerade versus masquerade: An unusual presentation of systemic B-cell lymphoma misdiagnosed as giant cell arteritis - A case report. Indian J Ophthalmol Case Rep 2022;2:494-6

How to cite this URL:
Abbas S, Lewis K, Rennie C. Masquerade versus masquerade: An unusual presentation of systemic B-cell lymphoma misdiagnosed as giant cell arteritis - A case report. Indian J Ophthalmol Case Rep [serial online] 2022 [cited 2022 May 19];2:494-6. Available from: https://www.ijoreports.in/text.asp?2022/2/2/494/342980

We present an unusual uniocular presentation of systemic diffuse large B-cell lymphoma. There were also lessons learned regarding masquerading presentations and pattern recognition.

  Case Report Top

An 80-year-old Caucasian female presented with 4 days of left-sided visual loss preceded by a 2-week history of anorexia, headache, pain on chewing, and right chin numbness. She was a glaucoma suspect, but was lost to follow-up 7 years ago. She had rheumatoid arthritis, hypertension, anemia, and hypercholesterolemia, for which she took methotrexate, folate, ferrous fumarate, amlodipine, and simvastatin.

Her visual acuity was 0.34 OD and counting fingers OS. There was a left relative afferent pupillary defect. Both eyes were uninflamed with no vitreous activity. Her intraocular pressures were 24 and 21 mmHg OD and OS, respectively. Orbital assessment was normal with no proptosis or abnormal oculomotility. Initial fundoscopy revealed cupped right disk and swollen left disk with cotton wool spots [Figure 1]. No other abnormalities of the retina or choroid were identified. Perimetry demonstrated a right superior arcuate defect and left superior altitudinal defect.
Figure 1: (Top) Left color fundus photograph demonstrating swollen disk and cotton wool spot. (Bottom) Right color fundus photograph demonstrating cupped disk

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Blood testing revealed Erythrocyte Sedimentation rate mm/hr (ESR) 45, C-Reactive Protein mg/dL (CRP) 60, Haemoglobin g/dL (Hb) 11.4 g/dL, platelets 227, White Cell Count x10^9/L (WCC) 7.8, Alkaline phosphotase U/L (ALP) 36, and Alanine aminotrasferase U/L (ALT) 187.

Considering the systemic symptoms and impression of ischemic optic neuropathy, a diagnosis of giant cell arteritis (GCA) was made in line with the American College of Rheumatolog y's criteria for diagnosis. Pulsed intravenous methylprednisolone 500 mg resulted in improvement of headache and jaw pain. However, an inferior exudative retinal detachment extending to the macula was noted the next morning secondary to recumbency overnight. Optical Coherence Tomography (OCT) confirmed these findings, and ultrasound B-scan did not identify any underlying choroidal masses [Figure 2].
Figure 2: Spectral domain OCT image demonstrating exudative retinal detachment involving the macula of the left fundus

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Further blood testing revealed normal serum Angiotensin converting enzyme (ACE), calcium, negative syphilis/lyme serology, negative Anti-nuclear antibody (ANA)/connective tissue screen/ Circulating Anti-Neutrophil Cytoplasmic Antibody (cANCA)/ Perinuclear antineutrophil cytoplasmic antibodies (pANCA), and equivocal quantiferon result, suggestive of immunocompromised state. Fundus fluorescein angiography showed retinal pigment epithelium (RPE) mottling, but did not identify any leakage, vasculitis, and any masses. Indocyanine green angiography showed good choroidal flow and excluded other choroidal pathology.

Considering the retinal detachment and chin numbness, alternative diagnoses were sought. Magnetic resonance imaging (MRI) of orbits and brain revealed ischemic changes within the left parietal and right occipital lobes and a diminished basilar artery, consistent with a posterior arteritis and possibility of posterior reversible encephalopathy syndrome aggravated by her methotrexate. Following rheumatology review, the diagnosis was still, however, felt to be GCA. A subsequent Magnetic Resonance Angiography (MRA), however, demonstrated lytic bone lesions within the skull and skull base, with the differential diagnosis of multiple myeloma or solid malignancy metastases. By day 4 of her admission, her vision deteriorated to hand movements, disk swelling increased, and the retinal detachment enlarged.

We enlisted support from hematology, oncology, rheumatology, and neurology. A radionuclide bone scan, CT chest/abdomen/pelvis, urinary Bence–Jones protein, and immunoglobulin electrophoresis were suggested to evaluate the presence of multiple myeloma or primary solid malignancy.

Prior to these investigations being conducted, however, our patient developed a mesenteric infarction. Her health rapidly declined and she sadly died hours later. The final diagnosis of diffuse large B-cell lymphoma was determined on postmortem. Her temporal artery biopsy was negative.

  Discussion Top

This case of diffuse large B-cell lymphoma demonstrates some very unusual ocular manifestations.

Exudative retinal detachment

Complement fixation via the alternative pathway and cytokine release by lymphoma cells have been linked to damage of RPE that could underpin exudative retinal detachment in the absence of evidence of direct choroidal spread.[1] This would explain the RPE mottling seen on the fundus fluorescein angiogram. One would expect the fellow eye to demonstrate similar changes and although healthy clinically, subtle RPE changes may have been missed due to the initial fluorescein run starting on the left. Alternatively, hypercoagulability secondary to hematologic malignancy may lead to partial occlusion of the choriocapillaris with resultant disturbances of Bruch's membrane and RPE layer with secondary subretinal exudation.[2],[3]

Most cases linking serous retinal detachments to systemic hematologic malignancies are bilateral[4],[5],[6] and mostly linked to acute lymphoblastic leukemia with evidence of direct choroidal infiltration on Indocyanine Green Angiography (ICG) or enhanced depth imaging OCT and/or masquerading as a panuveitis, in complete contrast to our case. There is only one other report of unilateral non-infiltrative serous retinal detachment related to non-Hodgkin's lymphoma in a patient with HIV and immunocompromised condition.[7] The authors postulated a systemic inflammatory disturbance as the underlying mechanism. Interestingly, that case also presented with facial numbness, jaw pain, and headache.

Disk swelling

Direct infiltration is the most likely reason for disk swelling in systemic lymphoma.[8] However, consistent with a generalized thrombotic tendency, cases of non-arteritic ischemic optic neuropathy with systemic lymphoma have been reported.[9] This could explain the combination of disk swelling, cotton wool spots, altitudinal field defect, and lack of visual response to systemic steroids in our case.

As clinicians, we regularly engage in type 1, automated thinking[3] that enables us to recognize patterns, make swift diagnoses, institute treatment, and efficiently process patients in busy clinics. Kahneman's insight into type 2 cognitive thinking[3] reminds us that pattern recognition can mislead and overlook important details.[10] GCA is more commonly encountered than vitreoretinal lymphoma in western eye clinics. It is also more rapidly devastating, necessitating a low threshold of suspicion and treatment. Although our patient had a combination of altitudinal field defect, swollen disk, headache, and raised ESR/CRP, consistent with GCA, she did not have true jaw claudication and had contralateral sensory deficits and an exudative retinal detachment, which did not fit with a diagnosis of GCA. The diagnosis of GCA became increasingly unlikely with further investigation, although the diagnosis was elusive until postmortem. Without her unexpected rapid demise, we may never have arrived at the correct diagnosis.

  Conclusion Top

To our knowledge, this is only the second documented case of unilateral serous retinal detachment in an eye without uveitis/pseudouveitic activity or choroidal infiltrate, which was due to underlying non-Hodgkin's lymphoma.

This case also reminded us of the pitfalls of pattern recognition and the need to scrutinize the pieces of the jigsaw that do not fit. In this case, it was two masquerading giants of the ophthalmologic world at play: GCA and lymphoma.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

  References Top

Yang P, Baciu P, Parker Kerrigan BC, Etheridge M, Sung E, Toimil BA, et al. Retinal pigment epithelial cell death by the alternative complement cascade: Role of membrane regulatory proteins, calcium, PKC, and oxidative stress. Invest Ophthalmol Vis Sci 2014;55:3012-21.  Back to cited text no. 1
Golan S, Goldstein M. Acute lymphocytic leukemia relapsing as bilateral serous retinal detachment: A case report. Eye 2011;25:1375-8.  Back to cited text no. 2
Kahneman D. 1934-author. Thinking, Fast and Slow. New York: Farrar, Straus and Giroux, 2011.  Back to cited text no. 3
Kurup SK, Levy-Clarke G, Calvo KR, Jaffe ES, Nussenblatt RB, Chan C-C. Primary diffuse large B-cell lymphoma of the spleen with coincident serous retinal detachments responsive to corticosteroids. Clin Exp Ophthalmol 2007;35:468-72.  Back to cited text no. 4
Lee CS, Shim JW, Yoon JS, Lee SC. Acute lymphoblastic leukemia presenting as bilateral serous macular detachment and lacrimal gland enlargement. Can J Ophthalmol 2012;47:e33-5.  Back to cited text no. 5
Moon SJ, Han DP. Acute bilateral serous macular detachment associated with gallium nitrate in a patient with non-hodgkin lymphoma. Retina 2005;25:212-4.  Back to cited text no. 6
Andreoli CM, Husain D, Davis TS, Loewenstein JI. Chorioretinal changes heralding metastatic malignancy. Arch Ophthalmol 2006;124:1790-2.  Back to cited text no. 7
Wong D, Danesh-Meyer H, Pon J-A. Infiltrative lymphomatous optic neuropathy in non-hodgkin lymphoma. J Clin Neurosci 2015;22:1513-5.  Back to cited text no. 8
Bassi ST, Dasgupta A. Bilateral simultaneous nonarteritic anterior ischemic optic neuropathy in a patient with alcoholic liver disease. Oman J Ophthalmol 2014;7:72-4.  Back to cited text no. 9
[PUBMED]  [Full text]  
Carbon C-C. Understanding human perception by human-made illusions. Front Hum Neurosci 2014;8:566.  Back to cited text no. 10


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