Cabugueira et al., J Clin Case Rep 2015, 5:8
DOI: 10.4172/2165-7920.1000579
Volume 5 • Issue 8 • 1000579
J Clin Case Rep
ISSN: 2165-7920 JCCR, an open access journal
Open Access Case Report
Case Report: Intraocular Non-Hodgkin Lymphoma
Ana Cabugueira*, Vanessa Lemos, Marco Dutra Medeiros, Rita Flores and Pinto Ferreira
Central Lisbon Hospital Center, NOVA Medical School, Universidade NOVA de Lisboa, Lisbon, Portugal
*Corresponding author: Ana Cabugueira, Central Lisbon Hospital Center, NOVA
Medical School, Universidade NOVA de Lisboa, Lisbon; Portugal, Tel: +351 21 371
5600; E-mail: anacabugueira@hotmail.com
Received April 25, 2015; Accepted August 06, 2015; Published August 13, 2015
Citation: Cabugueira A, Lemos V, Medeiros MD, Flores R, Ferreira P (2015)
Case Report: Intraocular Non-Hodgkin Lymphoma. J Clin Case Rep 5: 579.
doi:10.4172/2165-7920.1000579
Copyright: © 2015 Cabugueira A, et al. This is an open-access article distributed
under the terms of the Creative Commons Attribution License, which permits
unrestricted use, distribution, and reproduction in any medium, provided the
original author and source are credited.
Abstract
Purpose: Primary intraocular lymphoma is a primary central nervous system lymphoma in which lymphoma
cells invade the retina, vitreous, or optic nerve head without concomitant central nervous system involvement. The
aim of this presentation is to report a case of primary intraocular non-Hodgkin lymphoma.
Methods: The authors present a case report of a 77 years-old female with painless decreased in visual
acuity and foaters. Ophthalmic examination, Static computerized Perimetry, Spectral-Domain Optical Coherence
Tomography, angiography, laboratory study, lumbar puncture, computed tomography scan and magnetic resonance
imaging were performed.
Results: On examination, best corrected visual acuity was 20/25 in the right eye and 30/60 in the left eye. Slip
lamp examination revealed anterior chamber reaction and fundoscopy showed vitritis, optic disc edema, macular
edema and vasculitis in left eye. After three months of follow-up, the patient’s best corrected visual acuity decreased
to 20/60 in right eye and light perception in left eye. Fundoscopy revealed vitritis, optic disc edema, macular
edema and vasculitis in both eyes. A granular pattern, leakage from retinal vessels and optic disc were observed
in fuorescein angiography. Infectious and infammatory etiologies were excluded. Pars plana vitrectomy and retinal
biopsy were performed in left eye. Cytology evaluation revealed atypical lymphoid cells with large nuclei, prominent
nucleoli and basophilic cytoplasm, and confrmed the diagnosis of intraocular lymphoma.
Conclusion: Intraocular Lymphomas are rare malignancies that display a wide array of clinical manifestation,
therefore diagnosis can be challenging. It requires a high degree of clinical suspicion and differential diagnosis
includes infectious and non-infectious etiologies.
Keywords: Primary intraocular lymphoma; Central nervous system;
Fundoscopy; Vasculitis
Introduction
Primary Intraocular Lymphoma (PIOL) is a rare malignancy
of primary Central Nervous System (CNS) without concomitant
intracranial involvement. If there was vitreoretinal lymphoma with
CNS disease, this is classifed as Primary Central Nervous System
Lymphoma (PCNSL) [1]. In PIOL, usually, lymphoma cells invade
the retina, vitreous, or optic nerve head whereas secondary intraocular
lymphoma predominantly infltrates uveal tract, particularly the
choroid. PIOL, usually, presents as a difuse, large B-cell non-Hodgkin’s
lymphoma. Te etiology of PIOL and PCNSL are still unknown [1,2].
Te average age in immunocompetent patients is the late 50s and
60s and the rate appears to be increasing dramatically, especially in
immunocompromised patients [3].
Te diagnosis is difcult and ofen delay, because it can present
with a wide variety of manifestations, that can mimic many ocular
conditions [3]. Classic presentation is painless visual acuity decline
with foaters and chronic uveitis poorly responsive to corticosteroids
[4]. On Slit-lamp examination, anterior chamber cells are presented up
to 75% of cases. Te most frequent posterior segment signs are vitritis
and classic lesions, such large, multifocal, cream or yellow subretinal
infltrates. Tis lesions are considered pathogmonic of this disease.
Afer the resolution of this lesions a leopard-spot apperence may be
seen. Other manifestations are multiple white dots, retinal infltration,
retinal vasculitis, vein and arterial occlusion, fundus favimaculatus-
like, exsudative retinal detachment, choroidal involvement, optic disc
invasion [5].
Te diagnostic approach includes neurological evaluation, such
Computerized Tomography (CT), Magnetic Resonance Imaging
(MRI), Lumbar Pucntion (LP) and vitreous or chorioretinal biopsy.
60-80% of patients with PIOL develop cerebral lymphoma and
systemic spread is rare, occurring in only 6 to 8% of cases. Te average
time separating the onset of ocular symptoms and CNS disease is 29
months [3]. It requires a high degree of clinical suspicion and diferential
diagnosis includes infectious and non-infectious etiologies. 6 Optimal
treatment for PIOL and PCNSL with ocular involvement is controversial
and has yet to be defned. Te aim of our study was to describe a case of
a rare malignancy as PIOL and the diagnostic work-up.
Case Report
Te authors describe a case of a 77 years-old, caucasian female,
presented with painless decreased visual acuity and foaters in her Lef
Eye (LE) with fve days of evolution. In the frst visit, Best Corrected
Visual Acuity (BCVA) was 20/25 on the Right Eye (RE) and 20/60 on
the LE.
Slit-lamp examination revealed deep anterior chamber with +
2 tyndall. Intraocular pression was 16 mmHg and 15 mmHg in RE
and LE, respectively. Fundus examination showed vitritis, optic disc
swollen, macular edema and vacuities in the LE (Figure 1). Te patient
denied other neurological complains.
Systemic and laboratory evaluations including complete blood
count, erythrocyte sedimentation rate, C-reactive protein, tuberculin
skin test, angiotensin converting enzyme,urine analysis, chest X-ray,
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ISSN: 2165-7920