Cid J, Revilla M, Cervera A, Cervantes F, Muñoz E, Ferrer I, Montserrat E
Hematology Department, Hospital Clínic, IDIBAPS, Barcelona, Spain.
Ann Hematol. 2000 Jul;79(7):392-5. doi: 10.1007/s002779900149.
Progressive multifocal leukoencephalopathy (PML) is a subacute demyelinating disorder of the central nervous system usually affecting immunocompromised individuals and is due to infection of the oligodendrocytes by the JC virus. A case of PML in a chronic lymphocytic leukemia (CLL) patient treated with fludarabine is reported, representing the second such instance in which the diagnosis of the neurological disorder was established by brain biopsy. A 61-year-old man with a 14-year history of B-cell type CLL, for which he had received chlorambucil therapy 10 years earlier, developed progressive paresis of both left extremities at 7 months of receiving low doses of oral fludarabine, when his CD4 count has decreased to 0.08 x 10(9)/l. Cranial magnetic resonance imaging revealed a subcortical focal lesion at the right precentral gyrus and a focal lesion at the right thalamus, and a stereotactic brain biopsy showed pathological findings consistent with PML, namely severe myelin breakdown, reactive astrocytosis, and abnormal, huge glial cells with large bizarre nuclei showing granular basophilic inclusions, whereas the presence of the JC virus was demonstrated by in situ hybridization. The present case, in addition to a few previously reported, calls attention to the possibility that severe neurological side effects can be associated with the immunosuppression provoked by the use of fludarabine in CLL patients.
进行性多灶性白质脑病（PML）是一种中枢神经系统的亚急性脱髓鞘疾病，通常影响免疫功能低下的个体，由JC病毒感染少突胶质细胞所致。本文报道了1例接受氟达拉滨治疗的慢性淋巴细胞白血病（CLL）患者发生PML的病例，这是第二例通过脑活检确诊该神经系统疾病的病例。一名61岁男性，有14年B细胞型CLL病史，10年前曾接受苯丁酸氮芥治疗，在接受低剂量口服氟达拉滨7个月时出现双下肢进行性无力，此时其CD4细胞计数已降至0.08×10⁹/L。头颅磁共振成像显示右侧中央前回皮质下局灶性病变及右侧丘脑局灶性病变，立体定向脑活检显示病理结果符合PML，即严重髓鞘破坏、反应性星形细胞增生，以及异常的巨大神经胶质细胞，其核大且形态怪异，可见嗜碱性颗粒状包涵体，而原位杂交证实存在JC病毒。除了少数先前报道的病例外，本病例提醒人们注意，使用氟达拉滨治疗CLL患者引发的免疫抑制可能会伴有严重的神经副作用。
