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Successful eradication of leptomeningeal plasma cell disease

机译:成功根除软脑膜浆细胞疾病

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Plasma cell leukaemia (PCL) is a rare and aggressive form of malignant monoclonal gammopathy characterized by the presence of high levels of plasma cells in peripheral blood. Central nervous system involvement of PCL has no established treatment and an extremely poor prognosis. We here present a 59-year-old male patient diagnosed with PCL, initially treated with induction chemotherapy followed by autologous peripheral blood hematopoietic stem cell transplantation. After achieving a partial response, he relapsed and presented with leptomeningeal disease. He was then successfully treated with dexamethasone, pomalidomide, and an intrathecal combination of methotrexate, methylprednisolone and cytarabine. This cleared his cerebrospinal fluid from plasma cells achieving a durable partial response.
机译:浆细胞白血病(PCL)是一种恶性单克隆丙种球蛋白病的罕见且侵袭性形式,其特征是外周血中存在高水平的浆细胞。 PCL的中枢神经系统受累尚未建立治疗方法,并且预后极差。我们在这里介绍了一名59岁的男性患者,其诊断为PCL,最初接受诱导化疗,然后进行自体外周血造血干细胞移植。达到部分反应后,他复发并出现了软脑膜疾病。然后,他成功地地塞米松,泊马利度胺,以及甲氨蝶呤,甲基强的松龙和阿糖胞苷的鞘内联合治疗。这从浆细胞清除了他的脑脊髓液,实现了持久的部分反应。

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