Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0079731 (B-cell lymphoma)
 16,671 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Intraocular lymphoma is a rare ocular malignancy that may occur in the retina or the uvea. Retina or vitreoretinal lymphoma accounts for the majority of cases and is often secondary to diffuse large B-cell lymphoma. In the present study, a 66-year-old Caucasian male with a history of Waldenstrom's macroglobulinemia with diffuse large B-cell lymphoma, presented with blurred vision in the left eye one month following cycle 4 of an R-CHOP regimen. At the time of onset, the patient was being treated for bacterial pneumonia. Visual acuity was 20/25 in his right eye (OD) and 20/30 in the left (OS). Ophthalmologic examination showed intraretinal white infiltrates associated with hemorrhage in the superotemporal midperiphery of the retina and vitritis OS. Initial diagnostic considerations included infectious (cytomegalovirus retinitis, syphilis, toxoplasmosis, tuberculosis), inflammatory (retinal vasculitis associated with autoimmune disease or hypercoagulable states) or malignant (intraocular lymphoma) diseases. The patient did not respond to intravitreal injection of foscarnet and oral valgancyclovir. Systemic work-up and aqueous fluid biopsy were inconclusive. Diagnostic vitrectomy yielded inconclusive results and the patient continued to have progressive loss of vision. A repeat diagnostic vitrectomy with retinal and subretinal biopsy confirmed large B cells consistent with metastatic B-cell lymphoma. A concomitant PET/CT scan was performed that revealed bilateral new pulmonary nodules resulting in additional chemotherapy. Our case shows the diagnostic dilemmas in patients with systemic lymphoma and the possible role of concurrent systemic restaging in patients with ocular complaints, even when in systemic remission.
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PMID:Metastatic B-cell lymphoma masquerading as infectious retinitis and vasculitis. 2278 26

Primary vitreoretinal lymphoma (PVRL) is a rare malignancy that is speculated to arise extraocularly, and preferentially invade and flourish in the ocular and CNS microenvironments. The eye is involved in about 20% of primary central nervous system lymphomas, but the brain is eventually involved in about 80% of PVRL. Most are B-cell lymphomas with small numbers of T-cell lymphomas metastatic to the vitreous and retina. Metastatic systemic B-cell lymphoma usually involves choroid. Primary choroidal lymphoma is rare. Intraocular lymphoma can usually be distinguished from uveitis clinically, although there are overlaps, which may be pronounced in eyes with a large component of reactive inflammation related to tumor surveillance and control. There are controversies in diagnosis and treatment. Diagnosis through examination of ocular fluid is technically difficult and can utilize cytology, immunohistochemistry, flow cytometry, molecular detection of gene rearrangements, and cytokine profiling. Treatment of intraocular lymphoma without detectable CNS disease could consist of a full course of systemic chemotherapy with ocular adjunctive treatment, or ocular treatment alone depending on the preference of the clinical center. In ocular only cases where the vitreous has been debulked to improve vision and there is no sight-threatening involvement of the RPE, orbital irradiation or intravitreal chemotherapy stabilizes the intraocular process but does not seem to modify the CNS component, which can present symptomatically in an advanced state. This is a highly malignant disease with a poor prognosis. Close collaboration with a pathologist and oncologist, and good communication with patients is essential.
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PMID:Intraocular lymphoma: a clinical perspective. 2319 50

Intraocular lymphoma may occur, primarily with or without overt parenchymal CNS lymphoma or secondarily from a variety of other lymphomas. The diagnosis is frequently based on cytologic features and/or a limited panel of ancillary techniques. Twenty-seven cytology samples from the vitreous preparations of 20 patients with clinical suspicion or histopathologic diagnoses of lymphoma were retrospectively reviewed (2007-2011). Floaters and decreased visual acuity were the most common ocular symptoms. Sixteen (of 20) patients had at least one cytology that was "positive" or "suspicious" for lymphoma (F = 10; M = 6, median age = 71 years, range = 52-82). Involvement was limited to the eye in half of the cases (n = 8). Seven patients had CNS involvement, and three had systemic lymphoma. Cytologic specimens were classified as large B cell lymphoma (LBCL) (n = 13), suspicious for LBCL (n = 5), atypical/negative (n = 5), and consistent with NK-T cell lymphoma (n = 1). Three cytologic samples were acellular. Cytologic findings included increased cellularity (high n = 7, moderate n = 7), large to medium (n = 19) cell size, marked nuclear irregularities (n = 12), frequent apoptosis (n = 7), lymphoglandular bodies (n = 12), and necrosis (n = 12). Easily identifiable mitotic figures were present in two cases. CD20 immunocytochemistry performed in 15 cases with available cell blocks/destained cytospins preparations, was confirmatory in 8 cases. Cytologic evaluation of intraocular lymphoma is possible in vitreous specimens. Extraocular involvement was not present in half of the cases. The most useful cytologic features include increased cellularity, necrosis, and nuclear enlargement. Ancillary immunocytochemical studies are useful and support the morphologic impression in a subset of cases.
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PMID:Cytologic features in vitreous preparations of patients with suspicion of intraocular lymphoma. 2416 62