INTRAVASCULAR LYMPHOMATOSIS - AN INDOLENT OR AGGRESSIVE ENTITY

Intravascular lymphomatosis (IVL) is a rare malignancy characterized b y neoplastic proliferation of lymphoid cells within the lumens of arte ries, small veins and capillaries. We report four patients with IVL an d review the recent world literature, relating to incidence, clinical features and possible therapy. In these cases diagnosis was establishe d coincidentally in one patient after prostatectomy. This patient even tually had central nervous system involvement. In two other patients N L was diagnosed from skin lesions. In the fourth case the diagnosis wa s established at post-mortem examination, where involvement of most or gans was evident but particularly kidneys, myocardium, gastrointestina l tract and lymph nodes. Therapy was given to three patients, but the disease progressed in two and they both died with evidence of central nervous system involvement, while the third patient has had a good par tial response to combination chemotherapy but has relapsed within two months of completing chemotherapy. As evident from our patients and th e Literature review IVL has a variable clinical course and currently, there appears to be no effective therapy for this rare disorder.