Pseudouveitis

Abstract

Primary oculocerebral non-Hodgkin lymphoma (NHL) of the immunocompetent patient is associated with significant morbidity and mortality, but early diagnosis and follow-up may improve prognosis. The eye, anatomically and embryologically part of the central nervous system (CNS), can be the primary site of the lymphomatous process. In patients with symptoms of atypical uveitis, vitrectomy can be of great help for early diagnosis of primary central nervous system lymphoma. We retrospectively reviewed the diagnostic features, treatment, and evolution of 10 patients with primary central nervous system lymphoma who presented with symptoms of pseudouveitis. The patients complained of chronic vitreal opacities, increasing with time. These symptoms contrasted with the absence of the usual signs of inflammation of the anterior segment or of the retina, which characterize true uveitis. Vitrectomy was proposed after lumbar puncture and cerebral magnetic resonance imaging. Six vitrectomies were carried out, 3 patients had a stereotaxic biopsy, and 1 patient had a cardiac biopsy. A pathologic diagnosis of large B-cell lymphoma was made on vitrectomy specimens in 100% of the patients who had this procedure. The mean time from onset of ocular symptoms to diagnosis was 24 months. This series was characterized by a rare systemic dissemination of the NHL (negative in 80%), a strong preponderance of B-cell NHL, and the absence of association with Epstein-Barr virus (EBV) among these immunocompetent patients. To our knowledge, this series includes the only reported case of oculocardiac lymphoma. Meningeal dissemination appeared to be associated with a poor prognosis. Neurologic complications of treatment combining radiotherapy and methotrexate were significant among patients older than 60 years of age. The current study suggests that primary central nervous system lymphoma should be suspected in patients with pseudouveitis, and that the diagnosis can be established quickly and without side effects by vitrectomy. These patients should be followed carefully in order to detect meningeal dissemination.

Biology and Treatment of Primary Central Nervous System Lymphoma

“…Patients with isolated ocular disease may present with an insidious decrease in visual acuity and because this symptom is non-specific, there can be a long time lag between the onset of symptoms and diagnosis. 30, 31 Patients may also note vitreal opacities that increase over time 32 and visual hallucinations have been described. Spinal lesions are uncommon in PCNSL and they are typically manifest as discrete intramedullary nodules.…”

A sarcoidosis–lymphoma syndrome revealed by hypopituitarism

“…Interleukin-10 (IL-10) is a growth factor and differentiation factor for B-lymphocytes, while IL-6 is a multifunctional cytokine playing a role in host inflammatory defense mechanisms. Ocular lymphomas are therefore often associated with elevated levels of IL-10 relative to levels of IL-6 in the vitreous (12). This ratio was very low in the anterior chamber liquid of our patient, thus consistent with uveitis of inflammatory and non-neoplastic origin.…”