Aims/Purpose: Waldenstrom's macroglobulinemia (WM) is a rare hematological neoplasm characterized by lymphoplasmacytic proliferation, monoclonal immunoglobulin M (IgM) production, and bone marrow infiltration. Ocular involvement can be one of the signs of disease activity, and the purpose of this case is to present the ocular manifestations that may occur through a clinical case seen in consultation.Methods: A review was conducted of a case involving a 64‐year‐old male patient seen in the ophthalmology department of our hospital, with no known drug allergies and a personal history of Waldenstrom's macroglobulinemia diagnosed in 2018, currently in remission. The patient presented with progressive visual acuity loss in the left eye in the context of possible vitritis/chorioretinitis. He was treated with trimethoprim, sulfamethoxazole, and prednisone due to the suspicion of a possible infectious cause. Laboratory tests, serologies, a vitreous biopsy, fluorescein angiography, and ocular ultrasound were performed to determine the etiology. A few weeks later, he presented with clinical symptoms of disorientation and behavioral changes, prompting an emergency visit. A brain MRI was performed, revealing a tumoral lesion in the left frontal lobe, suggestive of cerebral lymphoma.Results: After studying and biopsying the brain lesion, the diagnosis of cerebral lymphoma was confirmed, with ocular involvement in the form of vitritis and chorioretinitis as a sign of disease reactivation.Conclusions: Waldenstrom's macroglobulinemia can manifest in various ways, and one of them can be through ocular activity signs such as uveitis (both anterior and posterior), vitritis, retinitis, and chorioretinitis. Therefore, it is necessary to conduct a thorough differential diagnosis through a complete medical history, laboratory tests, serologies, and imaging studies when suspecting a possible disease reactivation.
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