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At the time the article was created Bruno Di Muzio had no recorded disclosures.View Bruno Di Muzio's current disclosures
At the time the article was last revised Frank Gaillard had the following disclosures:
- Biogen Australia Pty Ltd, Investigator-Initiated Research Grant for CAD software in multiple sclerosis: finished Oct 2021 (past)
These were assessed during peer review and were determined to not be relevant to the changes that were made.View Frank Gaillard's current disclosures
Intravascular lymphoma (IVL), also known as intravascular lymphomatosis or intravascular large cell lymphoma or intravascular large B-cell lymphoma, corresponds to a rare variant of extranodal diffuse large B cell lymphoma that affects small and medium-sized vessels and has no specific clinical or laboratory findings. CNS and skin manifestations are the most common forms, however, any organ may be involved 1,2,6.
Intravascular lymphoma usually affects elderly patients in their 6th to 7th decades of life, with a male-to-female ratio of 1:2 3.
It has non-specific clinical features which 3,4,8:
other clinical features which tend to have ethnic predilections:
Western variant: more common CNS and cutaneous involvement
Asian variant: more common bone marrow involvement, splenic involvmement, hepatic involvement, and hemophagocytic lymphohistiocytosis
If CNS involvement, a mild to moderate elevation of CSF protein is usually present 4.
The reason why lymphoma cells tend to stay in the intravascular space in intravascular lymphoma is a consequence of the absence of CD29 (β1 integrin) and CD54 (ICAM-1) surface ligands, which probably disable them from diapedesis across the endothelium 2.
The diagnosis of intravascular lymphoma is made postmortem in over 50% of cases 2.
If there is CNS involvement, angiography (DSA), CT and MRI often show evidence of multiple vascular occlusions and stroke as non-specific multifocal abnormalities 4-6.
T2/FLAIR: hypersignal abnormalities in a dynamic pattern (resolution of some and the new appearance of others) 5
DWI/ADC: restriction areas in a dynamic pattern 5
SWI: cerebral microhemorrhages may be present 7
T1 C+ (Gd): a persistent mass-like enhancement may be noted in proximity to the T2 or DWI changes. Different patterns of parenchymal and meningeal enhancement may be seen 5,6
Treatment and prognosis
Intravascular lymphoma usually has a rapidly fatal outcome, with patient overall survival lasting only a few months. Intravascular lymphoma is sensitive to systemic chemotherapy, however, the treatment still remains suboptimal due to the rarity of this disorder and the difficulty to establish a diagnosis in time 2.
History and etymology
This condition was first described in Germany by L Pfleger and J Tappeiner in 1959 and designated as angioendotheliomatosis proliferans systemisata 4.
Entities which result in scattered multifocal brain lesions, particularly small infarcts and hemorrhages, and clinically associated with a rapidly
progressive dementia should be considered 4,5,9.
- 1. Jethwani DP, Yadav R, Chickabasaviah YT. A 58-year-old lady with progressive neurological syndrome: Presence of an intravascular lymphoma. Neurol India. 2015;63 (2): 225-9. doi:10.4103/0028-3886.156288 - Pubmed citation
- 2. Fonkem E, Lok E, Robison D et-al. The natural history of intravascular lymphomatosis. Cancer Med. 2014;3 (4): 1010-24. doi:10.1002/cam4.269 - Free text at pubmed - Pubmed citation
- 3. Gan LP, Ooi WS, Lee HY et-al. A case of large B-cell intravascular lymphoma in the brain. Surg Neurol Int. 2013;4 (1): 99. doi:10.4103/2152-7806.115709 - Free text at pubmed - Pubmed citation
- 4. Williams RL, Meltzer CC, Smirniotopoulos JG et-al. Cerebral MR imaging in intravascular lymphomatosis. AJNR Am J Neuroradiol. 1998;19 (3): 427-31. Pubmed citation
- 5. Baehring JM, Henchcliffe C, Ledezma CJ et-al. Intravascular lymphoma: magnetic resonance imaging correlates of disease dynamics within the central nervous system. J. Neurol. Neurosurg. Psychiatr. 2005;76 (4): 540-4. doi:10.1136/jnnp.2003.033662 - Free text at pubmed - Pubmed citation
- 6. Slone HW, Blake JJ, Shah R et-al. CT and MRI findings of intracranial lymphoma. AJR Am J Roentgenol. 2005;184 (5): 1679-85. doi:10.2214/ajr.184.5.01841679 - Pubmed citation
- 7. Richie M, Guterman E, Shah M, Cha S. Susceptibility-Weighted Imaging of Intravascular Lymphoma of the Central Nervous System. JAMA Neurol. 2022;79(1):86. doi:10.1001/jamaneurol.2021.4391 - Pubmed
- 8. Ponzoni M, Campo E, Nakamura S. Intravascular Large B-Cell Lymphoma: A Chameleon with Multiple Faces and Many Masks. Blood. 2018;132(15):1561-7. doi:10.1182/blood-2017-04-737445 - Pubmed
- 9. Pons-Escoda A, Naval-Baudin P, Velasco R, Vidal N, Majós C. Imaging of Lymphomas Involving the CNS: An Update-Review of the Full Spectrum of Disease with an Emphasis on the World Health Organization Classifications of CNS Tumors 2021 and Hematolymphoid Tumors 2022. AJNR Am J Neuroradiol. 2023. doi:10.3174/ajnr.a7795 - Pubmed