Four varied cases of intravascular lymphomatosis and a literature review.

BACKGROUND: Intravascular lymphoma (IVL) is an uncommon malignancy defined pathologically by neoplastic proliferation of lymphoid cells within the lumens of capillaries, small veins, and arteries, with little or no adjacent parenchymal involvement. The nature of IVL has been the subject of considerable controversy. Recent immunohistochemical studies have demonstrated clearly that the tumors bear the immunophenotype of neoplastic lymphoid cells of either B-cell or T-cell lineage. IVL commonly affects the central nervous system, resulting in progressive dementia and multiple neurologic deficits; skin is the second most common site of involvement, in the form of cutaneous plaques and nodules.

METHODS: In a retrospective review of all cases of non-Hodgkin's lymphoma 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. Standard fixation, tissue processing, sectioning, and hematoxylin and eosin 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; the third rapidly progressive dementia, nonlocalizing neurologic deficits, 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, heart, gastrointestinal tract, glomerulus, central nervous system, and skin. Malignant lymphoid cells marked as B-cells in all cases.

CONCLUSIONS: Unusual and interesting clinical presentations may occur in patients with IVL. The medical literature indicates that few cases are diagnosed ante mortem. Although isolated patients may respond favorably to chemotherapy, most will deteriorate rapidly and the diagnosis of IVL not contemplated until necropsy.