PDF(Pubmed)
Abstract：
Late onset neutropenia (LON) related to rituximab or rituximab plus chemotherapy is defined as an unexplained absolute neutrophil count of ≤1.5 × 109/L starting at least four weeks after the last rituximab administration. LON is infrequent and its pathophysiology remains unknown. There are no guidelines or consensus strategies for the optimal management of patients developing LON. The majority of the patients recover promptly with no specific treatment and only some cases need to be managed with granulocytic colony stimulating factor (G-CSF), usually with a rapid response. Here, we describe a 69-year-old patient with Waldenström\'s macroglobulinemia who presented a septic event in the context of severe LON after rituximab plus bendamustine. The diagnosed of agranulocytosis was established by bone marrow examination. Interestingly, anti-neutrophil antibodies bound to the patient\'s granulocytes were found suggesting an autoimmune mechanism. The patient did not respond to G-CSF but achieved a rapid response after high doses of intravenous immunoglobulins with full white blood cell recovery.
摘要：
与利妥昔单抗或利妥昔单抗联合化疗相关的迟发性中性粒细胞减少症（LON）定义为在最后一次利妥昔单抗给药后至少四周开始的无法解释的绝对中性粒细胞计数≤1.5×109/L。LON很少见，其病理生理学仍然未知。对于发展为LON的患者的最佳管理，没有指南或共识策略。大多数患者在没有特殊治疗的情况下迅速康复，只有一些病例需要用粒细胞集落刺激因子（G-CSF）治疗，通常是快速反应。这里,我们描述了一名69岁的Waldenström巨球蛋白血症患者,在利妥昔单抗加苯达莫司汀后出现严重LON的脓毒症事件.通过骨髓检查确定粒细胞缺乏症的诊断。有趣的是,发现与患者粒细胞结合的抗中性粒细胞抗体提示自身免疫机制。患者对G-CSF无反应，但在高剂量的静脉内免疫球蛋白和全白细胞恢复后获得了快速反应。
