Original Investigation | Clinical Sciences

Vitreoretinal Presentation of Secondary Large B-Cell Lymphoma in Patients With Systemic Lymphoma

Diva R. Salomão, MD1,2; José S. Pulido, MD1; Patrick B. Johnston, MD, PhD3; Irene Canal-Fontcuberta, MD1; Andrew L. Feldman, MD2
[+] Author Affiliations
1Department of Ophthalmology, Mayo Clinic, Rochester, Minnesota
2Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota
3Department of Hematology, Mayo Clinic, Rochester, Minnesota
JAMA Ophthalmol. 2013;131(9):1151-1158. doi:10.1001/jamaophthalmol.2013.334.
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Importance  Intraocular lymphomas represent a diverse group of hematologic malignant neoplasms involving different tissues within the eye. Predominant involvement of the retina and vitreous without uveal infiltration in systemic lymphoma, mimicking a primary vitreoretinal lymphoma, is extremely rare. Our study emphasizes the importance of systemic evaluation in addition to central nervous system evaluation in all patients with newly diagnosed vitreoretinal lymphoma.

Objective  To determine the incidence of secondary intraocular lymphoma presenting as vitreoretinal infiltrates without central nervous system involvement, mimicking primary vitreoretinal lymphoma in patients with systemic lymphoma.

Design  Retrospective review of all vitreous aspiration biopsy samples acquired because malignant neoplasm was suspected clinically that were obtained at our institution from January 1, 2000, through December 31, 2010. Review included patient clinical history, radiographic study findings, cytologic and/or histologic preparations, immunophenotypic study findings, treatment, and outcomes.

Setting  Mayo Clinic pathology database.

Participants  Fifty-five patients with vitreous specimens available for review.

Exposures  Vitreous aspiration biopsy.

Main Outcome and Measure  Confirmation of the diagnosis of diffuse large B-cell lymphoma (DLBCL).

Results  Of the 55 patients with vitreous specimens available for review, 3 (5%) had a DLBCL infiltration in the vitreous without any central nervous system involvement as a manifestation of systemic lymphoma. All 3 patients were men, aged 54, 66, and 73 years, and had blurred vision and floaters for several weeks before undergoing diagnostic vitrectomy. Ophthalmic examinations revealed clumps of vitreous cells but no choroidal involvement. One patient had no history of lymphoma; the diagnosis of vitreoretinal lymphoma was followed by DLBCL after a lymph node biopsy. The other 2 patients had low-grade B-cell lymphoma and chronic lymphocytic leukemia for 29 and 7 months before large-cell transformation in the eye. Patients were treated with systemic chemotherapy plus intraocular injections of rituximab or methotrexate sodium.

Conclusions and Relevance  Vitreoretinal symptoms of DLBCL in patients with systemic lymphoma may be more frequent than previously thought (5% in our study). Not all lymphomas with vitreoretinal involvement represent primary intraocular lymphomas; thorough ophthalmologic evaluation in patients with visual symptoms and complete staging in patients with documented ocular lymphoma are of utmost importance.

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Figure 1.
Flowchart Depicting the Selection of Study Patients and Specimens
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Figure 2.
Patient 1, With a Diagnosis of Large B-Cell Lymphoma

A, Slitlamp examination reveals the presence of vitreous aggregates. B, From the vitreous aspiration biopsy sample, a cytospin preparation shows large atypical lymphocytes with a high ratio of nuclei to cytoplasm and scant cytoplasm (Diff-Quick stain, original magnification ×600). C, The cell block shows a cellular specimen composed mostly of large atypical lymphocytes with prominent nucleoli (hematoxylin-eosin, original magnification ×600). D, Staining of these cells was positive for CD20 (anti-CD20, original magnification ×600). E, The cervical lymph node biopsy specimen reveals large B-cell lymphoma (hematoxylin-eosin, original magnification ×600). F, Optical coherence tomography reveals subretinal and retinal infiltrates.

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Figure 3.
Patient 3, With a Diagnosis of Chronic Lymphocytic Leukemia

A, Fundus photograph shows vitreous haziness. B, Optical coherence tomography reveals irregularities at the retinal pigment epithelium layer. C, The vitreous aspiration biopsy sample shows a cellular specimen represented by large atypical lymphocytes with prominent nucleoli (hematoxylin-eosin, original magnification ×600). Inset highlights the prominent nucleoli (original magnification ×1000). D, Staining of the cells was positive for CD20 (anti-CD20, original magnification ×600).

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