Acute Pontine Osmotic Demyelination Syndrome at Presentation of Lymphoma in Association With Hypoalbuminemia

MAYO CLINIC PROCEEDINGS(2023)

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摘要
A previously healthy young woman presented to the emergency department with fevers, dyspnea, headache, and weight loss. She was found to have pancytopenia, elevated liver function tests, splenomegaly, and a right heterogenous hypervascular ovarian mass. Because of headaches and subacute hearing loss, magnetic resonance imaging of her brain was performed, which showed a trident-shaped symmetric nonenhancing T2-hyper-, T1-hypointensity with restricted diffusion in the central pons with sparing of the periphery, compatible with acute osmotic demyelination syndrome (ODS) (Figure 1).1Ruzek K.A. Campeau N.G. Miller G.M. Early diagnosis of central pontine myelinolysis with diffusion-weighted imaging.AJNR Am J Neuroradiol. 2004; 25: 210-213PubMed Google Scholar Lumbar puncture was unremarkable. Labs were notable for a serum albumin of 2.2 g/dL (reference range, 3.5 - 5.0 g/dL) but no electrolyte derangements. She underwent fine needle aspiration of the right ovary and bone marrow biopsy, which showed a high–grade B cell lymphoma with MYC and BCL6 rearrangements. She was treated with polatuzumab-vedotin, rituximab, cyclophosphamide, doxorubicin, and prednisone. After cycle 3 of therapy, she underwent re-staging imaging, which showed improvement in the pontine T2-hyperintensity (Figure 2) and expected resolution of the restricted diffusion (not shown). Her positron emission tomography–computer tomography scan also showed complete resolution of the lymphoma (not shown). This case highlights the unique imaging findings of ODS. Osmotic demyelination syndrome without clearly attributable symptoms has been rarely described in the setting of lymphoma without electrolyte derangements.2Doni E. Tremolizzo L. Patassini M. Pioltelli P.E. Ferrarese C. Appollonio I. Asymptomatic central pontine myelinolysis without hyponatriemia in diffuse large B cell lymphoma.Neurol Sci. 2016; 37: 2035-2037Crossref Scopus (6) Google Scholar Previous case reports of ODS in lymphoma have also occurred in the setting of severe hypoalbuminemia, and this osmotic stress may be a predisposing factor.3Nguyen P.C. Dunstan M. Kumar B. Low M.S.Y. Rare case of diffuse large B-cell lymphoma presenting with central pontine myelinolysis.BMJ Case Rep. 2021; 14e242924Crossref Scopus (1) Google Scholar,4Kawata E. Isa R. Yamaguchi J. et al.Diffuse large B-cell lymphoma presenting with central pontine myelinolysis: a case report.J Med Case Rep. 2015; 9: 131Crossref Scopus (5) Google Scholar This case, along with prior case reports, suggests that in some cases of lymphoma, ODS can be a presenting feature. The authors report no potential competing interests. The authors thank Thomas E. Witzig, MD, for his assistance with review of the case and manuscript preparation.
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osmotic demyelination,lymphoma,syndrome
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