Abstract
HIV-negative progressive multifocal leukoencephalopathy (PML) has a poor prognosis due to a lack of standard treatment. Herein, we report a patient with HIV-negative PML which occurred after the treatment for classical Hodgkin's lymphoma (CHL). A 71-year-old male patient was admitted to our hospital due to various neurological symptoms, including memory disturbance, dysgraphia, ataxia, and ideomotor apraxia, at 16 months after high-dose salvage chemotherapy with autologous peripheral blood stem cell transplantation (PBSCT) for primary treatment-refractory CHL. The patient's blood and serological examination results were mainly normal, including CD4-positive T lymphocyte count and serum immunoglobulin levels. T2-weighted fluid-attenuated inversion recovery MRI showed high-intensity lesions from the left occipital lobe to the corpus callosum. Moreover, the rapid intraoperative pathological assessment of biopsy specimens obtained from abnormal brain lesions suggested brain relapse of CHL. The patient's symptoms progressed rapidly; therefore, treatment with high-dose methotrexate was started, which significantly improved the patient's symptoms and MRI findings within a week. However, further examinations of the biopsy specimens with in situ hybridization and immunohistochemical examinations showed reactivation of the John Cunningham virus (JCV) in the astrocytes. Further, cells initially believed to be Hodgkin cells based on the rapid intraoperative pathological assessment were found to be destructive astrocytes, thereby confirming the diagnosis of PML. The patient was then successfully treated with combined mefloquine and mirtazapine and did not have any fatal outcomes. Based on this case, a differential diagnosis of PML from CNS involvement of CHL is important even in cases without evident biomarkers for immunodeficiency. Moreover, methotrexate was likely to be effective in improving neurological symptoms by decreasing brain parenchyma inflammation in the acute phase in this particular patient.