Pseudo‐hypopyon as the presenting feature in B‐cell and T‐cell intraocular lymphoma

Abstract

The clinicopathologic correlation of two patients with primary intraocular lymphoma is described, both of whom had a rare and unusual presentation of hypopyon uveitis. Sampling of aqueous fluid proved the cytopathologic diagnosis of B-cell lymphoma in the first patient. In the second patient an iris biopsy confirmed a T-cell lymphoma. The samples were examined using haematoxylin and eosin, and immunohistochemical stains. These patients were diagnosed with primary intraocular lymphoma on initial presentation with hypopyon uveitis. Secondary glaucoma was noted in the patient with T-cell lymphoma. Both cases appeared confined to the eye and initially responded favourably to aggressive chemotherapy and radiotherapy, but later went on to develop systemic involvement. Clinicians should be aware of this dramatic mode of presentation, which is unusual for ocular lymphoma.

Molecular pathology of lymphoma

“…1 Rare subtypes include T-cell-rich B-cell lymphoma 58 and T-cell lymphoma. [59][60][61][62] VRL are characterized by a subretinal or perivascular retinal infiltration of pleomorphic medium-to-large sized cells with minimal basophilic cytoplasm, indented or folded nuclei, and prominent, often multiple, nucleoli. These changes can be quite discreet (Figure 3).…”

Primary intraocular lymphoma: a review of the clinical, histopathological and molecular biological features

“…Intraocular lymphoma of T-cell type is less common than B-PIOL, and most cases represent an extension of mycosis fungoides (primary cutaneous T-cell lymphoma) or secondary manifestations of a systemic T-cell lym-DOI 10.1007/s00417-004-0973-0 phoma in conjunction with systemic leukaemia (ATL/L) and is associated with human T-cell lymphotropic virus type-1 (HTLV1) infection or with acquired immunodeficiency syndrome [12,18,25,33,39,52,61,66,71,72,79,84,87,88,92,94,96,99,101,119,120,126,134,135,[147][148][149][150][151]. Only a few cases of PIOL of T-cell origin without cutaneous or systemic involvement have been reported [31,79,99,146].…”

Primary intraocular lymphoma of T-cell type: report of a case and review of the literature

“…In two of the three described cases of intraocular T-cell lymphoma without systemic or CNS lymphoma the patients were alive, with follow-up of 6 and 18 months, at the time of reporting [6,10]. The third patient [48] presented with uveal thickening and a pseudo-hypopyon. There was no presence of systemic disease at the time of diagnosis, and tonsillar T-cell lymphoma developed only 5 years later.…”

“…Very few cases of T-cell-related primary intraocular lymphoma (T-PIOL) without any association with mycosis fungoides have been reported [33,38,48]. We present a case of T-PIOL with demonstration of a monoclonal rearrangement of the T-cell-receptor gamma (TCR-γ) gene.…”