Intravascular Large Cell Lymphoma
Clinical
- Rare, no incidence figures available
- Age
- Predominantly 7th and 8th decade
- Cases reported in 12 and 13 year olds
- One congential case reported but inconclusive phenotype (only CD43 positive with focal LCA reactivity)
- Geographic distribution
- Most cases reported from North America and Europe
- Asian variant primarity reported from Asia
- Sites of involvement
- Central nervous system and skin are the most commonly reported sites
- Brain lesions may present as dementia or as focal mass lesions
- Skin lesions may be nodules or plaques
- Disease limited to skin reported in 26%
- All females
- Better prognosis
- Wide range of other sites reported
- Liver and spleen in 26%
- Lymph nodes only in 11%
- Peripheral blood and marrow
- Older literature rarely reported involvement
- Recent reports using immunologic or genotypic means have found involvement
- Marrow frequently involved in Asian variant
- May be associated with autoimmune disorders
- Originally considered nearly uniformly fatal (most cases diagnosed at autopsy)
- Antemortem diagnosis with aggressive treatment may yield good results
