Ja. Digiuseppe et al., INTRAVASCULAR LYMPHOMATOSIS - A CLINICOPATHOLOGICAL STUDY OF 10 CASESAND ASSESSMENT OF RESPONSE TO CHEMOTHERAPY, Journal of clinical oncology, 12(12), 1994, pp. 2573-2579
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.