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Intravascular Lymphoma presenting stroke and ischemic myelopathy

RESUMO

Case presentation: A 78-year-old female presented with subacute and
progressive lower limb weakness and numbness, urinary retention, confusion and
weight loss. Her past medical history included hypertension, trigeminal neuralgia and
meningioma resection eleven years before presentation. Upon admission, neurological
examination showed impaired attention and executive dysfunction, along with
asymmetric spastic paraparesis (worse on the right leg) and a dermatomal sensory
deficit level at T4.
Laboratory findings were noticeable for pancytopenia. Patient underwent
neuroimaging. Brain MRI revealed multiple areas of T2/FLAIR hyperintensities in
addition with restricted diffusion in the parasagittal gray and white matter bilaterally,
overlapping with internal and external watershed zones of the frontal, parietal, and
occipital lobes, indicating an ischemic etiology. Spine MRI showed hyperintensities
on T2-weighted sequences, predominantly in the central and upper segments of
thoracic spinal cord, also consistent vascular insult. CSF examination was normal.
Because of pancytopenia, a bone marrow biopsy was obtained, which confirmed an
intravascular non-Hodgkin B cell lymphoma. Chemotherapy was initiated afterwards
and patient is showing gradual neurologic improvement during follow-up.
Discussion: Intravascular large B cell lymphoma (IVLBCL) is a
rare subtype of extranodal large B-cell lymphoma with an aggressive course, which
may affect multiples organs, including central nervous system. Its main feature
resides on the fact that tumor cells have a confined proliferation within the lumen of
small and medium-sized vessels, differentiating IVLBCL form other forms of lymphoma.
Even though any organ may be involved, skin and CNS are the most commonly
affected sites. Most frequent neurological symptoms are cognitive impairment
(encephalopathy and dementia) and myelopathy with neuroimaging showing infarct-like
lesions due to vessel occlusion by tumor cells. IVLBCL poses a diagnostic challenge
because of its protean manifestation and lack of specific laboratory and imaging findings.
In addition, PET-CT is usually negative because this type of tumor tends to remain
intravascular.
Final comments: The importance of this case lies in the fact that the combination of concomitant brain and
spinal cord scattered infarction with systemic findings (pancytopenia and constitutional
symptoms) must include this rare, underecognized disease in the differential diagnosis.

Palavras Chave

Stroke; Cerebrovascular disease; Lymphoma