Case of the Week
Section Editors: Matylda Machnowska1 and Anvita Pauranik2
1University of Toronto, Toronto, Ontario, Canada
2BC Children's Hospital, University of British Columbia, Vancouver, British Columbia, Canada
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June 15, 2015
Intravascular Large B-Cell Lymphoma (IVL)
- Background: IVL is a rare subtype of extranodal diffuse large B-cell lymphoma, with predilection for the CNS and skin. It is characterized by intravascular B-cell proliferation. The estimated incidence is < 1/1,000,000 per year.
- Clinical Presentation: It is nonspecific and includes changes in mental status, nonlocalizing neurologic deficits, seizures, fever of unknown origin, or skin changes. Peripheral blood involvement is seen in 5–9% of patients.
- Key Diagnostic Features: Premortem diagnosis is difficult because of its nonspecific clinical and imaging presentation. Immunologic analysis and electron microscopy are used. Brain and/or skin biopsy is used to confirm diagnosis before starting treatment. CT and MRI, although nonspecific, often show multiple infarct-like lesions due to small vessel occlusions.
- The MRI appearance is variable depending on the size of the affected vessels and the degree of associated inflammation. Findings include infarct-like lesions, metachronous nonspecific white matter lesions on T2WI, hemorrhagic infarctions, meningeal enhancement, masslike lesions, and hiperintense lesions in the pons on T2WI.
- DDx:
- Cerebral vasculitis
- Cerebrovascular disease
- Infection
- Neoplasia (gliomatosis cerebri may have similar clinical and imaging findings)
- Treatment: Chemotherapy by using R-CHOP. It has a high (> 80%) overall mortality rate and a mean survival of only 9–13 months.
