Abstract |
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.
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Authors | J Roy, C Ryckman, V Bernier, R Whittom, R Delage |
Journal | Leukemia
(Leukemia)
Vol. 12
Issue 7
Pg. 1026-30
(Jul 1998)
ISSN: 0887-6924 [Print] England |
PMID | 9665185
(Publication Type: Case Reports, Journal Article, Review)
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Topics |
- Adult
- B-Lymphocytes
(pathology)
- Biopsy
- Female
- Gene Rearrangement
- Genes, bcl-2
- Humans
- Immunohistochemistry
- Lymph Nodes
(pathology)
- Lymphocytosis
(complications, genetics, pathology)
- Lymphoma, B-Cell
(etiology, genetics)
- Lymphoma, Large B-Cell, Diffuse
(etiology, genetics, pathology)
- Polymerase Chain Reaction
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