Large cell lymphoma complicating persistent polyclonal B cell lymphocytosis.
Persistent polyclonal B cell lymphocytosis (PPBL) is a rare lymphoproliferative 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 after an initial diagnosis of PPBL; her disease was rapidly fatal despite intensive chemotherapy and blood stem cell transplantation. Because we had recently identified multiple bcl-2/Ig gene rearrangements in blood mononuclear cells of patients with PPBL, we sought evidence of this oncogene in this particular patient: bcl-2/Ig gene rearrangements were 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 lymphoproliferative disorder. Despite serologies consistent with past exposure to this virus, it was not found in lymphoma cells using a sensitive polymerase chain reaction technique. We conclude that non-Hodgkin's lymphoma may occur during the course of PPBL. However, longer follow-up in more patients will be needed in order to better clarity the risk of hematologic malignancy in patients with PPBL.
Division of Hematology, Saint Sacrement Hospital, Quebec City, Canada.
J
J
Roy
C
C
Ryckman
V
V
Bernier
R
R
Whittom
R
R
Delage
D000328:Adult; D001402:B-Lymphocytes / Q000473:pathology*; D001706:Biopsy; D005260:Female; D015321:Gene Rearrangement; D019254:Genes, bcl-2; D006801:Humans; D007150:Immunohistochemistry; D008198:Lymph Nodes / Q000473:pathology; D008218:Lymphocytosis / Q000150:complications* / Q000235:genetics / Q000473:pathology; D016393:Lymphoma, B-Cell / Q000209:etiology* / Q000235:genetics; D016403:Lymphoma, Large B-Cell, Diffuse / Q000209:etiology* / Q000235:genetics / Q000473:pathology; D016133:Polymerase Chain Reaction