persistent polyclonal B cell lymphocytosis (PPBL) is a rare lymphoprol
iferative disorder of unclear natural history and its potential for B
cell malignancy remains unknown. We describe the case of a 39-year-old
female who presented with stage IV-B large cell lymphoma 19 years aft
er an initial diagnosis of PPBL; her disease was rapidly fatal despite
intensive chemotherapy and blood stem cell transplantation. Because w
e had recently identified multiple bcl-2/lg gene rearrangements in blo
od mononuclear cells of patients with PPBL, we sought evidence of this
oncogene in this particular patient: bcl-2/Ig gene rearrangements wer
e found in blood mononuclear cells but not in lymphoma cells. Owing to
the possible role of Epstein-Barr virus (EBV) in the pathogenesis of
PPBL, we also hypothesized our patient might have an EBV-related lymph
oproliferative disorder. Despite serologies consistent with past expos
ure to this virus, it was not found in lymphoma cells using a sensitiv
e polymerase chain reaction technique. We conclude that non-Hodgkin's
lymphoma may occur during the course of PPBL. However, longer follow-u
p in more patients will be needed in order to better clarify the risk
of hematologic malignancy in patients with PPBL.