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Case Reports
. 2003 Jun;56(6):468-70.
doi: 10.1136/jcp.56.6.468.

Intravascular lymphomatosis

P C W Lui  1 , G K C Wong, W S Poon, G M K Tse
Affiliations
Case Reports

Intravascular lymphomatosis

P C W Lui et al. J Clin Pathol. 2003 Jun.

Abstract

Intravascular lymphomatosis (IVL) is a rare angiotrophic large cell lymphoma producing vascular occlusion of arterioles, capillaries, and venules. Antigenic phenotyping shows that these lymphomas are mostly of B cell type, and less commonly T cell or Ki-1 lymphomas. The central nervous system and skin are the two most commonly affected organs; patients usually present with progressive encephalopathy with mental status changes and focal neurological deficits and skin petechia, purpura, plaques, and discolouration. Other involved organs include adrenal glands, lungs, heart, spleen, liver, pancreas, genital tract, and kidneys. Bone marrow, blood, cerebrospinal fluid, and lymph nodes are typically spared. Fever of unknown origin is another common presentation. Only one case of IVL presenting with disseminated intravascular coagulation and anasarca (generalised oedema) has been reported in the literature. This report describes a postmortem case of a patient with IVL who initially presented with disseminated intravascular coagulation complicated by intracerebral haemorrhage.

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Figures

Figure 1
Figure 1
Photomicrographs showing (A) areas of haemorrhagic infarcts within the cerebral cortex, and (B) the presence of atypical lymphoid cells within the infarcts.
Figure 2
Figure 2
Photomicrographs showing (A) lymphoma cells within the surface small vessels over the cerebral cortex, and (B) that the cells are CD20 positive.
See this image and copyright information in PMC

References

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