4 VARIED CASES OF INTRAVASCULAR LYMPHOMATOSIS AND A LITERATURE-REVIEW

Background. Intravascular lymphoma (IVL) is an uncommon malignancy def ined pathologically by neoplastic proliferation of lymphoid cells with in the lumens of capillaries, small veins, and arteries, with little o r no adjacent parenchymal involvement. The nature of IVL has been the subject of considerable controversy. Recent immunohistochemical studie s have demonstrated clearly that the tumors bear the immunophenotype o f neoplastic lymphoid cells of either B-cell or T-cell lineage. IVL co mmonly affects the central nervous system, resulting in progressive de mentia and multiple neurologic deficits; skin is the second most commo n site of involvement, in the form of cutaneous plaques and nodules. M ethods. In a retrospective review of all cases of non-Hodgkin's lympho ma seen at our institution, four cases of IVL were identified. Case 1 was fixed in methacarin, and Cases 2-4 were fixed in 10% formalin. Sta ndard fixation, tissue processing, sectioning, and hematoxylin and eos in staining were used. Immunophenotypic studies were performed using a modified avidin-biotin complex technique. The specimen in Case 1 was stained by Giemsa stain using standard techniques. Results. Four cases of IVL are presented. One patient experienced hypoxemia and fevers of unknown origin; the second, dementia and a vasculitislike illness; th e third rapidly progressive dementia, nonlocalizing neurologic deficit s, and panhypopituitarism; the fourth Kaposi-like skin lesions. Case 1 relapsed shortly after completing chemotherapy. Salvage chemotherapy has yielded good initial response. Autopsy findings in cases 2, 3 and 4 confirmed IVL in many vessels, including the brain, lung, liver, hea rt, gastrointestinal tract, glomerulus, central nervous system, and sk in. Malignant lymphoid cells marked as B-cells in all cases. Conclusio ns. Unusual and interesting clinical presentations may occur in patien ts with IVL. The medical literature indicates that few cases are diagn osed ante mortem. Although isolated patients may respond favorably to chemotherapy, most will deteriorate rapidly and the diagnosis of IVL n ot contemplated until necropsy.