Bilateral Granulomatous Panuveitis as Initial Presentation of Diffuse Systemic T Cell Lymphoma

Abstract

A high-grade diffuse T cell lymphoma, initially simulating bilateral panuveitis, was diagnosed by analysis of a vitreous biopsy specimen and a breast tumor in a 57-year-old woman. It responded favorably to aggressive chemotherapy before it relapsed in leukemic transformation. This case emphasizes the misleading initial symptoms of primary intraocular lymphoma and the role of immunophenotyping in the diagnosis and classification of lymphoproliferative ocular disorders. The presentation and management of uveal lymphoid neoplasia are discussed.

Intraocular lymphoma: a series of 14 patients with clinicopathological features and treatment outcomes

“…Of 23 published cases with clinical data, [17][18][19][20][21][22][23][24][25][26][27][28][29][30][31][32][33][34][35] nine (39%) were associated with mycosis fungoides (primary T-cell malignancy of skin), 20,24,25,27,[29][30][31][32]34,35 10 (43%) with systemic (nonmycosis) lymphoma, [17][18][19]22,23,[26][27][28]33 two (9%) with CNS and intraocular lymphoma only, 17,20 and two (9%) were restricted to the eye only. 18,21 Therefore, systemic disease is found in 82% of patients with T-cell intraocular This was the total dose administered to the cranial vault and orbits.…”

“…24,25,31,32,34 Of those with systemic (nonmycosis) T-cell lymphoma, two-thirds of reported patients have died with a median survival of 8 months after diagnosis of the eye disease. 22,23,[27][28]33 However, two patients with documented systemic T-cell lymphoma are still alive with long follow-up of 60 and 95 months. 17,18 The two reported cases of intraocular T-cell lymphoma without systemic or CNS lymphoma were alive with a relatively short follow-up of 6 and 18 months.…”

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

“…According to the literature, those patients with an intraocular lymphomatous manifestation with underlying mycosis fungoides usually have a relatively long history of cutaneous lymphoma (median 7 years), and died within weeks to months of developing intraocular lymphoma [22,23,25,32,42,47,49,69,70]. Of those with systemic (non-mycosis) T-cell lymphoma, the majority of reported patients died within a year after diagnosis of intraocular disease, although exceptional cases have been described (up to 95 months survival) [6,10,18,32,33,35,38,44,57,61,72]. 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].…”

Ocular manifestations as first signs of systemic T cell lymphoma in two cases

“…Systemic lymphomas usually metastasize though blood into the uveal tissues [1]. Ocular manifestations include vitritis, posterior uveitis with distinct yellowish subretinal epithelium infiltrates and occasionally anterior uveitis and optic nerve involvement [2]. Here we reported two cases of uveitis patients finally diagnosed as systemic T cell lymphoma.…”