Department of Hematology, Okayama City Hospital, 3-20-1 kitanagaseomote, Okayama-shi, Okayama, Japan.
Department of Pathology, Okayama City Hospital, 3-20-1 kitanagaseomote, Okayama-shi, Okayama, Japan.
Int J Hematol. 2023 Jan;117(1):137-142. doi: 10.1007/s12185-022-03449-y. Epub 2022 Sep 6.
Primary effusion lymphoma-like lymphoma (PEL-LL) shows a unique clinical presentation, characterized by lymphomatous effusions in the body cavities. PEL-LL may be associated with hepatitis C virus infections and fluid overload states; and owing to its rarity, no standard therapies have been established. We report a case of a 55-year-old woman who developed PEL-LL during treatment with dasatinib, for chronic myeloid leukemia (CML). She presented to our hospital with dyspnea lasting for approximately a month and showed pericardial and bilateral pleural effusions. The pericardial effusion was exudative, and cytopathological and immunophenotypic examinations showed numerous CD 20-positive, large atypical lymphoid cells, which were also positive for the Epstein-Barr virus gene. No evidence of lymphadenopathy or bone marrow infiltration was found. We diagnosed PEL-LL, immediately discontinued dasatinib, and performed continuous drainage of the pericardial effusions. Complete response was achieved, and remission was maintained for 15 months. Two months after discontinuation of dasatinib, she was administered imatinib and a deep molecular response for the CML was maintained. PEL-LL occurring during dasatinib treatment is rare. We compared the results of previous reports with this case, and found that early diagnosis of PEL-LL, discontinuation of dasatinib, and sufficient drainage can improve the prognosis of PEL-LL.
原发性渗出性淋巴瘤样淋巴瘤(PEL-LL)表现出独特的临床特征,表现为体腔中的淋巴瘤性渗出。PEL-LL 可能与丙型肝炎病毒感染和液体超负荷状态有关;由于其罕见性,尚未建立标准的治疗方法。我们报告了一例 55 岁女性在接受达沙替尼治疗慢性髓性白血病(CML)期间发生 PEL-LL 的病例。她因呼吸困难持续约一个月就诊于我院,并出现心包和双侧胸腔积液。心包积液为渗出性,细胞病理学和免疫表型检查显示大量 CD20 阳性、大而异常的淋巴样细胞,这些细胞还对 EBV 基因呈阳性。未发现淋巴结病或骨髓浸润的证据。我们诊断为 PEL-LL,立即停用达沙替尼,并进行心包积液的持续引流。完全缓解,缓解期持续 15 个月。停用达沙替尼两个月后,给予伊马替尼治疗,CML 维持深度分子反应。达沙替尼治疗期间发生的 PEL-LL 罕见。我们将本病例与以往的报告结果进行了比较,发现早期诊断 PEL-LL、停用达沙替尼和充分引流可以改善 PEL-LL 的预后。