ABSTRACT
Background: Recognizing vitreous haze (VH) patterns on ultra-widefield imaging (UWFI) in nonresolving vitritis can reduce delay in suspecting vitreoretinal lymphoma (VRL) and in performing an early vitreous biopsy for definitive diagnosis. Purpose: To demonstrate role of UWFI in providing a clue for suspecting VRL in case of bilateral nonresolving dense vitritis and demonstrate precautions for high yield of lymphoma cells on vitreous biopsy. Synopsis: A 52-year-old healthy phakic lady came with gradual, painless blurred vision OS>OD for 6 months.Treated elsewhere for OU vitritis with steroids (local and systemic), anti-tubercular therapy for 2 months, and azathioprine, she had no improvement. Presenting best-corrected visual acuty was counting fingers OD and hand movement OS. Anterior segment OU was quiet. Fundus showed OD 3+ and OS 4+ vireous haze (VH). UWFI showed 揳urora borealis� pattern of VH (OS>OD) and 搒tring of pearls� OD. Ultrasonography B-scan OU showed complete posterior vitreous detachment and attached retina. OU VRL was suspected. MRI brain and orbit with contrast was found to be normal. After stopping steroids for 2 weeks, OS underwent pars plana vitrectomy (PPV) and intravitreal (IV) methotrexate 400 mcg/0.1 ml + rituximab 1mg/0.1ml. Vitreous sample sent for cytology and immunohistochemistry showed diffuse large B-cell non-Hodgkins lymphoma with CD20 and MUM1-positive cells. OD underwent two IV rituximab injections at monthly interval initially followed by PPV with IV rituximab and methotrexate. The patient remained in remission during close follow-up. Recognizing VH patterns on UWFI can reduce the delay in the diagnosis of VRL and early initiation of treatment. Highlights: VH patterns in VRL depend on state of vitreous liquefaction and syneresis.Aurora borealis pattern on UWFI results from linear opacities with lymphoma cells uniformly aligned along formed vitreous fibrils.String of pearls pattern results from clumps of lymphoma cells and inflammatory material over the scaffold of vitreous fibrils.These patterns provide high index of suspicion for considering VRL as d iagnosis.IV rituximab has minimal side effects and has been effective in managing VRL with isolated ocular involvement.
ABSTRACT
Vitreoretinal lymphoma(VRL)is a rare and aggressive non-Hodgkin's lymphoma, and its early and correct diagnosis is still a great challenge because of its non-specific clinical presentation. For VRL diagnosis, pathological cytology is still the gold standard, but its diagnosis needs to combine with clinical manifestations, imaging features, immunological and molecular technology and so on. With the advancement of diagnostic technology, more efficient techniques of cytology and assistant diagnosis have been explored. Cytokines and interleukin score for intraocular lymphoma diagnosis(ISOLD), myeloid differentiation gene 88(MYD88)mutation and next-generation sequencing have higher diagnostic accuracy, so they have gradually become important auxiliary diagnostic methods and research hotspots.
ABSTRACT
Purpose: To present challenging cases of vitreoretinal lymphoma (VRL) that was misdiagnosed as uveitis because of the apparent intraocular inflammation. At the light of the new classification of intraocular lymphomas, we detail the characteristics that masqueraded the tumors and the clinical aspects that guided us to the correct diagnosis. Materials and Methods: We retrospectively reviewed the patients referred to our uveitis service between January 2006 and December 2014. Results: Seven patients referred with a presumptive diagnosis of idiopathic uveitis received a final diagnosis of VRL. The median time between the onset of symptoms and definitive diagnosis was 25 months for these complex cases. The median time from presentation at our clinic to final diagnosis was 1 month. The described clinical features including dense vitreous cells and subretinal infiltrates were characteristic and tend to be present in all these chronically ill patients. Vitreous samples were collected, and all demonstrated the pathognomonic tumor cells, the specific immunoglobulin heavy chain gene rearrangements, and an interleukin (IL)‑10 to IL‑6 ratio >1. Conclusion: VRLs are severe diseases with a poor prognosis that may be misdiagnosed as idiopathic inflammatory conditions of the eye. Treatment with steroids may occult the tumors and delay the correct diagnosis. Appropriate evaluation may prompt to a timely vitreous sampling and therefore to a faster diagnosis in these peculiar cases where the correct diagnosis was delayed by several months.