Synonyms:
a.k.a. neoplastic angioendotheliosis a.k.a. angiocentric lymphoma a.k.a. angiotropic malignant lymphoma
Diagnosis:
Suggested by MRI and angiography, and confirmed by pathology (CSF, brain biopsy)
Clinical features:
- Encephalopathy, dementia, seizures
- Strokes, myelopathy
- Can affect any organ: most commonly CNS & Skin or fever of unknown origin
Pathology:
- NonHodgkin lymphoma, B-cell
- Confined to blood vessels only, doesn’t extend into brain
- Blood vessels: in white & grey matter & Subarachnoid vessels
- Immunohistochemistry: CD20 positive B cells
Findings on Investigations:
CT:
- May be normal
MRI:
- Multifocal infarcts
- Focal parenchymal enhancement may occur, dural enchancement
- Nonspecific white matter lesions
Catheter Angiogram: may have appearance of vasculitis
CSF:
- Pleocytosis, increased protein
- Oligoclonal bands positive
- Rarely cytology positive
Other tests:
- ESR: high