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Intravascular lymphoma presenting as transverse myelitis in a patient with myelodysplastic syndrome
EP33149
Poster Title: Intravascular lymphoma presenting as transverse myelitis in a patient with myelodysplastic syndrome
Submitted on 14 Oct 2020
Author(s): Christian A. Linares, Tanveer Hasan, Hnin Hnin Aung
Affiliations: Guy's and St Thomas' NHS Foundation Trust, East Kent Hospitals University NHS Foundation Trust, Sherwood Forest Hospitals NHS Foundation Trust
This poster was presented at BIR Annual Congress 2020
Poster Views: 135
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Poster Information
Abstract: Background: Intravascular lymphoma (IVL) is a rare subtype of large B-cell lymphoma that often presents with neurological signs. Diagnosis of IVL is notoriously difficult and often not established until post-mortem examination.

Case presentation: Here we describe the case of a 72-year-old male with a history of myelodysplastic syndrome (MDS), transitional cell carcinoma and ischaemic heart disease who presented with acute-onset, progressive spastic paraparesis localisable to the spine with MRI and laboratory findings that pointed strongly to a diagnosis of idiopathic transverse myelitis (TM). After treatment with corticosteroids failed to provide durable improvement, a workup for a potential occult intramedullary spinal cord malignancy led to random skin biopsies that revealed underlying IVL, which has been shown to mimic TM.

Discussion: MDS commonly progresses to acute myeloid leukaemia (AML) but is not known to give rise to lymphoid malignancies, despite a few reports of IVL following a diagnosis of MDS. In patients with suspected TM, MRI is crucial for excluding compressive myelopathy but is insufficient for diagnosing IVL, and whenever IVL is suspected, diagnosis should be attempted by random skin biopsies before a brain or spine biopsy, even in the absence of overt skin findings. Early diagnosis is critical for getting patients on appropriate chemotherapy, so IVL should always be on the differential diagnosis in patients presenting with apparent TM, with or without MDS.
Summary: Here we describe the case of a 72-year-old male with intravascular lymphoma (IVL) of the spine that was masked as transverse myelitis (TM) and confounded by an existing diagnosis of myelodysplastic syndrome (MDS). IVL should be considered in patients presenting with progressive focal neurological symptoms, with or without MDS. Whenever IVL is suspected, diagnosis should be attempted first by random skin biopsies before considering a brain or spine biopsy, even without overt skin manifestations.References: 1. Arber DA, Orazi A, Hasserjian R, Thiele J, Borowitz MJ, Le Beau MM, et al. The 2016 revision to the World Health Organization classification of myeloid neoplasms and acute leukemia. Blood 2016; 127: 2391–405.
2. Valencia-Sanchez C. Transverse myelitis. BMJ Best Practice. June 2020. https://bestpractice.bmj.com/topics/en-gb/1061. Accessed 08 July 2020.
3. Pawar NH, Loke E, Aw DC. Spinal Cord Infarction Mimicking Acute Transverse Myelitis. Cureus 2017; 9: e1911.
4. Flanagn EP, Keegan BM. Paraneoplastic myelopathy. Neurol Clin 2013; 31: 307–18.
5. Transverse Myelitis Consortium Working Group. Proposed diagnostic criteria and nosology of acute transverse myelitis. Neurology 2002; 59: 499–505.
6. Sips GJ, Amory CF, Delman BN, Kleinman GM, Lipsey LR, Tuhrum S. Intravascular lymphomatosis of the brain in a patient with myelodysplastic syndrome. Nat Rev Neurol 2009; 5: 288–92.
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