Intravascular lymphomatosis presenting as systemic inflammatory response syndrome

Intravascular lymphomatosis is an unusual form of non-Hodgkin lymphoma characterized by intravascular proliferation of atypical lymphoid cells in multiple organs. It can cause systemic inflammatory response syndrome due to primary release of cytokines by the tumor cells or secondary release of cytokines after vascular occlusion by the tumor cells. It is a potentially fatal condition, because multiorgan failure can ensue due to thrombotic vascular occlusion. This is a very rare condition and most cases are diagnosed post mortem. We present a case of systemic inflammatory response syndrome and subsequent death from multiorgan failure in a patient with intravascular lymphoma.