Intravascular lymphomatosis. A report of three cases

Intravascular lymphomatosis (IVL) is a rare malignancy characterized by a proliferation of atypical lymphoid cells occluding small blood vessels (venules, capillaries and small arteries). The symptoms are caused by embolisms due to massive proliferation. Nervous system and skin are the most common sites of involvement but all organs may be involved, although it is typified by the absence of malignant cells in lymphoid tissues. We describe three cases of IVL: first patient was a 57 years old man with rapidly progressive dementia and neurological involvements and second case of a 69 years old man hospitalised with predominant symptoms in the lung (cough, dyspnea and fever), and a woman presenting as fever of unknown origin (FUO) with systemic inflammatory response syndrome. In all cases that we reported have been diagnosed post-mortem because of rapid progression of a multisystem disease and the absence of pathognomonic clinical manifestations. Diagnosis can be made using biopsy of one of the involved organs. In conclusion, we propose that IVL should be included in the differential diagnosis of acute confusional state, dementia or other unexplained neurological manifestations, fever of unknown origin, vasculitis, occult neoplasia or infections with signs of a systemic disease and marked elevation of serum lactate dehydrogenase (LDH).