Diagnosis of intravascular lymphoma by a novel biopsy site

Purpose To report the first known case of intravascular lymphoma (IVL), a rare disease, diagnosed via lacrimal gland biopsy. Design Interventional case report. Participants Single patient case report. Intervention Bedside lacrimal gland biopsy. Main Outcome Measures Clinicopathologic diagnosis of IVL, a neoplasm with only a few prior reports of ophthalmic manifestations, via a novel biopsy site. Results A 70-year-old Chinese man with 6 months of progressive paraplegia complained of blurred vision in both eyes. He had unremarkable cerebrospinal fluid studies and magnetic resonance imaging that showed abnormal thoracic cord signal and periventricular brain white matter changes with a normal pituitary gland and stalk. Dilated fundus examination showed multifocal areas of intra- and subretinal hemorrhage with serous retinal detachment. The workup included serologies and 2 normal bone marrow biopsies but did not reveal the underlying etiology. Because of the continued high suspicion for a malignant process, we performed bedside transconjunctival biopsy of the lacrimal gland. This demonstrated large, atypical CD20-positive B cells confined to the vessel lumina, consistent with IVL, an unusual form of large B-cell lymphoma. Conclusions This case represents the first time that IVL has been detected via lacrimal gland biopsy, which may be a useful way to investigate cryptogenic neurologic processes. Furthermore, it is one of the only reported cases of ophthalmic IVL diagnosed before autopsy. IVL is a rare disease but has protean manifestations involving the central nervous system, skin, bone marrow, liver, and spleen. The eye should also be considered an end organ for involvement.