Intravascular lymphoma is a rare form of non-Hodgkin lymphoma characterized by almost exclusive growth of neoplastic large lymphocytes within blood vessel lumina. Intravascular lymphoma is morphologically characterized in most instances by a B-cell phenotype, although rare cases with T- or NK-cell phenotype have been reported. This lymphoma is an aggressive and usually disseminated disease that predominantly affects elderly patients, resulting in poor performance status, B-symptoms, anemia, and a high serum lactate dehydrogenase level. The brain and skin are the most commonly involved sites. The usually disappointing survival after conventional chemotherapy has been recently improved by the introduction of immunotherapy (anti-CD20 antibody, rituximab). Differences in clinical presentation exist, mainly due to the association of IVL with the hemophagocytic syndrome. Intensive chemotherapy combinations containing drugs with higher central nervous system bioavailability are needed in cases with brain involvement.
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