SYSTEMIC INFLAMMATORY RESPONSE SYNDROME IN INTRAVASCULAR LYMPHOMATOSIS

Intravascular lymphomatosis (IVL) is characterized by an intravascular proliferation of atypical mononuclear cells of haematopoietic origin occluding small blood vessels including venules, capillaries and small arteries, and can affect virtually any organ in the body. Clinically, the most common manifestations are involvement of the skin and centra l nervous system, although various organ involvement has been describe d. We report a patient who presented with fever and rash, and succumbe d with acute neurological symptoms and systemic inflammatory response syndrome (SIRS) with no evidence of infection, The postmortem revealed disseminated involvement of virtually every organ with IVL, the malig nant cells being of T-cell origin, which may secrete cytokines includi ng tumour necrosis factor and interleukin-1, that are believed to be m ediators in SIRS. In the absence of evidence of infection on microbiol ogy and pathology, we postulate that the features of SIRS in this pati ent was due to the disseminated malignancy itself.