Bruserud Øyvind, Hansen Bent-Are, Vetti Nils, Johansen Silje, Reikvam Håkon
Section for Endocrinology, Department of Clinical Science, University of Bergen, Bergen, Norway.
Department of Microbiology, Førde Hospital, Førde, Norway.
Oxf Med Case Reports. 2018 Jul 3;2018(7):omy038. doi: 10.1093/omcr/omy038. eCollection 2018 Jul.
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.
浆细胞白血病（PCL）是一种罕见且侵袭性的恶性单克隆丙种球蛋白病，其特征是外周血中存在高水平的浆细胞。PCL累及中枢神经系统尚无既定的治疗方法，预后极差。我们在此报告一名59岁男性患者，诊断为PCL，最初接受诱导化疗，随后进行自体外周血造血干细胞移植。在达到部分缓解后，他复发并出现软脑膜疾病。然后他成功接受了地塞米松、泊马度胺以及甲氨蝶呤、甲基泼尼松龙和阿糖胞苷鞘内联合治疗。这使他的脑脊液中浆细胞清除，实现了持久的部分缓解。
