Department of Pathology, Duke University Medical Center, Durham, NC 27710, USA.
Hum Pathol. 2010 Dec;41(12):1802-8. doi: 10.1016/j.humpath.2010.04.019. Epub 2010 Sep 24.
A small fraction of patients with chronic lymphocytic leukemia/small lymphocytic lymphoma develop Epstein-Barr virus-positive B-cell lymphoproliferative disorders. These Epstein-Barr virus-B-cell lymphoproliferative disorders are thought to be related to immune suppression induced by fludarabine/other chemotherapeutic regimens. As in other immunodeficiency-associated lymphoproliferative disorders, these disorders demonstrate a heterogeneous histological spectrum that ranges from polymorphic to monomorphic to classical Hodgkin lymphoma-like lesions. We report a case of concurrent classical Hodgkin lymphoma and plasmablastic lymphoma in a patient with chronic lymphocytic leukemia/small lymphocytic lymphoma treated with fludarabine. Both classical Hodgkin lymphoma and plasmablastic lymphoma were positive for Epstein-Barr virus-encoded RNA, whereas classical Hodgkin lymphoma was also positive for Epstein-Barr virus- latent membrane protein 1, suggesting a different viral latency. Immunoglobulin gene rearrangement studies demonstrated distinct clones in the plasmablastic lymphoma and chronic lymphocytic leukemia/small lymphocytic lymphoma. These findings suggest biclonal secondary lymphomas associated with iatrogenic immunodeficiency. Epstein-Barr virus-B-cell lymphoproliferative disorders in the setting of chronic lymphocytic leukemia/small lymphocytic lymphoma, in particular those arising after chemotherapy, should be separated from true Richter's transformation, and be categorized as (iatrogenic) immunodeficiency-associated lymphoproliferative disorder.
一小部分慢性淋巴细胞白血病/小淋巴细胞淋巴瘤患者会发展为 EBV 阳性 B 细胞淋巴增生性疾病。这些 EBV-B 细胞淋巴增生性疾病被认为与氟达拉滨/其他化疗方案引起的免疫抑制有关。与其他免疫缺陷相关的淋巴增生性疾病一样,这些疾病表现出从多形性到单形性到经典霍奇金淋巴瘤样病变的异质性组织学谱。我们报告了一例在接受氟达拉滨治疗的慢性淋巴细胞白血病/小淋巴细胞淋巴瘤患者中同时发生经典霍奇金淋巴瘤和浆母细胞淋巴瘤的病例。经典霍奇金淋巴瘤和浆母细胞淋巴瘤均为 EBV 编码 RNA 阳性,而经典霍奇金淋巴瘤也为 EBV 潜伏膜蛋白 1 阳性,提示不同的病毒潜伏期。免疫球蛋白基因重排研究显示浆母细胞淋巴瘤和慢性淋巴细胞白血病/小淋巴细胞淋巴瘤中存在不同的克隆。这些发现提示与医源性免疫缺陷相关的双克隆继发性淋巴瘤。慢性淋巴细胞白血病/小淋巴细胞淋巴瘤中 EBV-B 细胞淋巴增生性疾病,特别是化疗后发生的疾病,应与真正的里希特转化区分开来,并归类为(医源性)免疫缺陷相关的淋巴增生性疾病。