Primary intraocular lymphoma.

Primary intraocular lymphoma (PIOL) is an ocular malignancy that is a subset of primary central system lymphoma (PCNSL). Approximately one-third of PIOL patients will have concurrent PCNSL at presentation, and 42-92% will develop PCNSL within a mean of 8-29 months. Although rare, the incidence has been rising in both immunocompromised and immunocompetent populations. The majority of PIOL is diffuse large B-cell lymphoma, though rare T-cell variants are described. Recently, PIOL has been classified by main site of involvement in the eye, with vitreoretinal lymphoma as the most common type of ocular lymphoma related to PCNSL. Diagnosis remains challenging for ophthalmologists and pathologists. PIOL can masquerade as noninfectious or infectious uveitis, white dot syndromes, or occasionally as other neoplasms such as metastatic cancers. Laboratory diagnosis by cytology has been much aided by the use of immunocytochemistry, flow cytometry, biochemical finding of interleukin changes (IL10:IL6 ratio > 1), and cellular microdissection with polymerase chain reaction amplification for clonality. Use of several tests improves the diagnostic yield. Approaches to treatment have centered on systemic methotrexate-based chemotherapy, often with cytarabine (Ara-C) and radiotherapy. Use of intravitreal chemotherapy with methotrexate (0.4 mg/0.1 mL) is promising in controlling ocular disease, and intravitreal rituximab (anti-CD20 monoclonal antibody) has also been tried. Despite these advances, prognosis remains poor.

Collagen VI assemblies in age-related macular degeneration.

Age-related macular degeneration (AMD) is the most common cause of incurable blindness in the developed world. Little is known about the pathogenesis of this condition, but deposits in Bruch's membrane and immediately beneath the retinal pigment epithelium are frequent findings associated with this disease. Within these deposits, molecular assemblies with an approximately 100-nm axial periodicity are seen. Two types of assembly are present: one exhibiting transverse double bands of protein density that are 30nm apart and repeat axially every approximately 100nm; the other with transverse double bands of protein density, 30nm apart and repeating axially every approximately 50nm. In this second type of assembly, more prominent pairs of bands alternate with less prominent ones. By comparison with analogous aggregates found in the vitreous of a patient with a full-thickness macular hole, collagen VI was singled out as the most probable protein constituent of the AMD aggregates. Possible models for the aggregation patterns of these assemblies are discussed in terms of collagen VI dimers and tetramers. Understanding the structure and chemical composition of the assemblies within the AMD basal deposits may prove of great help in understanding the pathophysiology of AMD itself.