Roy J, Ryckman C, Bernier V, Whittom R, Delage R
Division of Hematology, Saint Sacrement Hospital, Quebec City, Canada.
Leukemia. 1998 Jul;12(7):1026-30. doi: 10.1038/sj.leu.2401040.
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.
持续性多克隆B细胞淋巴细胞增多症（PPBL）是一种罕见的淋巴细胞增殖性疾病，其自然病程尚不清楚，其发展为B细胞恶性肿瘤的可能性也未知。我们描述了一例39岁女性患者，她在最初诊断为PPBL 19年后出现IV-B期大细胞淋巴瘤；尽管接受了强化化疗和造血干细胞移植，她的病情仍迅速恶化并导致死亡。由于我们最近在PPBL患者的血液单核细胞中发现了多个bcl-2/Ig基因重排，我们在该特定患者中寻找这种癌基因的证据：在血液单核细胞中发现了bcl-2/Ig基因重排，但在淋巴瘤细胞中未发现。由于爱泼斯坦-巴尔病毒（EBV）在PPBL发病机制中可能起作用，我们还推测该患者可能患有EBV相关的淋巴细胞增殖性疾病。尽管血清学检查结果表明患者既往接触过这种病毒，但使用敏感的聚合酶链反应技术在淋巴瘤细胞中未检测到该病毒。我们得出结论，非霍奇金淋巴瘤可能在PPBL病程中发生。然而，需要对更多患者进行更长时间的随访，以便更好地明确PPBL患者发生血液系统恶性肿瘤的风险。
