Multicentric Castleman's disease (MCD) is a lymphoproliferative disorder th
at can be defined based upon both clinical and pathological characteristics
. The clinical features of this frequently fatal disease include fever, gen
eralized lymphadenopathy, fatigue, splenomegaly, hepatomegaly, and pancytop
enia, Recently, severe forms of this disease have been diagnosed in HIV pos
itive patients. Human herpesvirus type 8 (HHV-8) DNA sequences have been de
tected in peripheral blood mononuclear cells (PBMCs) of patients with Kapos
i's sarcoma and MCD, regardless of HIV infection status, Treatment and outc
omes in HIV associated MCD are generally unfavorable, We recently treated t
wo HIV-positive patients diagnosed with aggressive MCD with daily oral etop
oside (50 mg). The first patient had relapsed on several occasions despite
previous therapy with doxil, paclitaxel, and oral ganciclovir. The second p
atient was treatment naive. Both patients had HHV-8 detectable by polymeras
e chain reaction in PBMCs, widespread tumor, and B-type symptoms when thera
py was initiated. In both cases remissions (documented by computerized tomo
graphy) have been durable, 1.5 and 6 months, respectively, with minimal sid
e effects. Oral etoposide may be a safe, tolerable, and active agent in MCD
. (C) 2001 Wiley-Liss, Inc.