Describe cases of intravascular lymphoma (IVL) that presented with transverse myelitis (TM). 

IVL is a neoplastic disorder where lymphoid cells are restricted to lumina of vessels, which results in constricted blood flow and thereby infarction. Central nervous system is most common affected organ with encephalopathy being primary symptom.  Unfamiliarity with IVL and its presentation has resulted in most cases being diagnosed at autopsy.

Case series and literature review

Case (1) Seventy-seven-year-old male presented with paraparesis. MRI showed TM from T10 to conus. Patient responded to pulse steroids, but then declined. Autoimmune, metabolic and infectious markers were unrevealing. Cerebrospinal fluid (CSF) showed elevated protein, elevated IgG index, 13 matched oligoclonal bands (OCBs) and negative flow cytometry/cytology. Serology displayed anemia, thrombocytopenia, low HDL as well as elevated LDH, ferritin, and Beta-2-microglobulin.  MRI showed areas of restricted diffusion in brain. Random skin biopsies confirmed IVL with atypical intravascular CD20+ lymphocytes. Family pursued comfort measures given functional status.

Case (2):  Sixty-eight-year-old female presented with subacute paraparesis. MRI of spine showed a 4.5 mm hyperintense lesion in right lateral cord at T6. Patient had sensory improvement with pulse steroids, but then worsened. MRI brain revealed restricted diffusion in multiple vascular territories. Autoimmune, infectious and neoplastic markers were unrevealing. Serology displayed anemia, low HDL, elevated LDH and elevated Ferritin.  CSF was normal including IgG index, OCBs, and flow cytometry/cytology.  Random skin biopsies were unremarkable. Brain biopsy confirmed IVL with intravascular CD20+ lymphocytes. Patient was treated with rituximab, cyclophosphamide, hydroxydaunorubicin and prednisone.