This 70-year-old patient presented with confusion, weakness, and recent stroke-type symptoms. Brain MRI showed scattered contrast-enhancing white matter lesions involving the right and left cerebral hemispheres. Clinical considerations included vasculitis and CADASIL. Skin and muscle biopsy was performed.
What is the most likely diagnosis for this patient’s central nervous symptoms based on Figures #1 – #4?
B. Sickle cell disease
C. Intravascular lymphoma
Correct Answer: Intravascular lymphoma
The findings are consistent with the presence of Intravascular Lymphoma (IVL), B-cell immunophenotype as indicated by positive staining for CD20 and absence of staining for CD3. Similarly involved small blood vessels were present in this patient’s skin biopsy.
IVL is a relatively rare aggressive extranidal non-Hodgkin lymphoma with predominantly intravascular location. Central nervous system and skin are commonly involved, although other organ systems can be also be affected. Signs and symptoms are related to vascular obstruction and thrombosis.
- Seegobin K, et al. Clinical characteristics, prognostic indicators, and survival outcomes in intravascular lymphoma: Mayo Clinic experience (2003-2018). Am J Hematol. 2022 Sep;97(9):1150-1158. doi: 10.1002/ajh.26635. Epub 2022 Jul 6. PMID: 35713565; PMCID: PMC9541514.
- Shimada K, Kiyoi H. Current progress and future perspectives of research on intravascular large B-cell lymphoma. Cancer Sci. 2021 Oct;112(10):3953-3961. doi: 10.1111/cas.15091. Epub 2021 Aug 9. PMID: 34327781; PMCID: PMC8486207.
- Fonkem E, et al. Neurological presentations of intravascular lymphoma (IVL): meta-analysis of 654 patients. BMC Neurol. 2016 Jan 16;16:9. doi: 10.1186/s12883-015-0509-8. PMID: 26849888; PMCID: PMC4744383.
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