Le Dû Katell, Delaunay Jacques, Voldoire Maud, Cuvier Thomas, Renard Pierre-Yves, Quilichini Benoît, Wafflart Eric, Dubreuil Julien, Sadot-Lebouvier Sophie
Department of Hematology, Hôpital Privé Le Confluent, Nantes, France.
Department of Onco-hematology, Hôpital Privé Le Confluent, Nantes, France.
CNS Oncol. 2025 Dec;14(1):2563981. doi: 10.1080/20450907.2025.2563981. Epub 2025 Sep 26.
Cerebral lesions are rare in hairy cell leukemia (HCL), and its incidence remains to be determined. Identifying the cause can be challenging. In this report, we present a case of brain lesions occurring several years after diagnosis. A 76-year-old male patient presented to the Emergency Department with confusion. He had been diagnosed with HCL in 1999 and had received five lines of treatment. Cerebral imaging revealed multiple nodular lesions, with edema and a hemorrhagic appearance. Cerebrospinal fluid tests were negative. The tumor origin was retained due to concomitant relapse (blood, lymph nodes). Despite the partial efficacy of rituximab-cladribine treatment, the patient died of pneumonia. A review of the literature (PubMed, CrossRef, Google Scholar) identified seventeen cases between 1966 and 2024, with a median age of 59 years (33-80). Cladribine, with or without rituximab, was the most widely prescribed treatment regimen with a complete response rate of 57%. Four (23.5%) patients died (two from infection, one from gastrointestinal bleeding and one from an unknown cause). These atypical presentations suggest that brain imaging and advanced biological investigations should be performed to guide management.
脑病变在毛细胞白血病(HCL)中较为罕见,其发病率尚待确定。确定病因可能具有挑战性。在本报告中,我们介绍了一例在诊断数年后出现脑病变的病例。一名76岁男性患者因意识模糊就诊于急诊科。他于1999年被诊断为HCL,并接受了五线治疗。脑部影像学检查显示多个结节性病变,伴有水肿和出血表现。脑脊液检查为阴性。由于同时出现复发(血液、淋巴结),肿瘤起源得以保留。尽管利妥昔单抗-克拉屈滨治疗有部分疗效,但患者死于肺炎。对文献(PubMed、CrossRef、谷歌学术)的回顾发现,1966年至2024年间有17例病例,中位年龄为59岁(33-80岁)。含或不含利妥昔单抗的克拉屈滨是最常用的治疗方案,完全缓解率为57%。4例(23.5%)患者死亡(2例死于感染,1例死于胃肠道出血,1例死因不明)。这些非典型表现提示应进行脑部影像学检查和先进的生物学检查以指导治疗。