CASE REPORT

Intravascular lymphomatosis

P C W Lui¹, G K C Wong², W S Poon² and G M K Tse³

¹ Department of Pathology, United Christian Hospital, Hong Kong
² Department of Surgery, Division of Neurosurgery, Prince of Wales Hospital, Chinese University of Hong Kong, Shatin, NT, Hong Kong
³ Anatomical and Cellular Pathology, Prince of Wales Hospital

Correspondence to:
Correspondence to:
Dr G M K Tse, Department of Anatomical and Cellular Pathology, Prince of Wales Hospital, Shatin, NT, Hong Kong;
garytse{at}cuhk.edu.hk

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.

Keywords: intravascular lymphomatosis; malignant angioendotheliomatosis; disseminated intravascular coagulation

Abbreviations: DIC, disseminated intravascular coagulation; IVL, intravascular lymphomatosis; LCA, leucocyte common antigen

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