INTRAVASCULAR LYMPHOMATOSIS - A CLINICOPATHOLOGICAL STUDY OF 10 CASESAND ASSESSMENT OF RESPONSE TO CHEMOTHERAPY

Purpose: We report a clinicopathologic study of 10 cases of intravascu lar lymphomatosis (IVL) seen at a single institution, and assess the r esponse to chemotherapy in these patients, as well as those collected from a literature review. Patients and Methods: The clinical, patholog ic, and immunophenotypic features of 10 cases of IVL diagnosed at the Johns Hopkins Hospital since 1977 were studied. Follow-up information was obtained in each case by consultation with the treating physician. In addition, cases of IVL reported previously in which patients were treated with chemotherapy and for which follow-up data were available at the time of publication were reviewed. Results: In the present seri es of 10 cases, the most common clinical features were fever of unknow n origin (FUO), mental status changes, and rash. Diagnostic specimens were obtained from a variety of sources, including brain, skin, prosta te, liver, kidney, and gall bladder. All of the four patients treated with combination chemotherapy are alive and two have achieved long-ter m survival (48 and 45 months, respectively); the remaining two are ali ve and in complete remission (CR) after short follow-up duration of 6 months. Among 35 patients reported in the literature who received chem otherapy (including four from this series), 43% attained a CR and were free of disease at the time of publication. None of the three patient s in our series who received localized therapy (surgery with or withou t radiation therapy) is alive (mean survival duration, 9 months). For the three patients diagnosed at postmortem examination, the mean inter val between onset of symptoms and death was 3 months, and disease was widespread. Conclusion: These findings suggest that IVL represents a h igh-grade non-Hodgkin's lymphoma (NHL) with a propensity for systemic dissemination, and that CR and long-term survival may result in patien ts treated with aggressive combination chemotherapy. (C) 1994 by Ameri can Society of Clinical Oncology.