title: Long-term survival in a patient with progressive multifocal leukoencephalopathy after therapy with rituximab, fludarabine and cyclophosphamide for chronic lymphocytic leukemia.
abstract: A 50-year-old male with chronic lymphocytic leukemia (CLL) was treated with fludarabine, cyclophosphamide and rituximab, which produced a complete remission. Eight months after the last dose of rituximab he had visual disturbance, diminished muscular strength in the right arm and vesicular-papular lesions in the left ophthalmic branch region of the V cranial nerve. These were initially interpreted as herpes virus encephalopathy (HVE), but brain magnetic resonance imaging (MRI) showed evidence of demyelination consistent with progressive multifocal leukoencephalopathy (PML). Cerebrospinal fluid (CSF) analysis was negative for varicella zoster virus (VZV) and John Cunningham virus (JCV) DNA. The clinical suggestion of PML prompted us to perform a brain biopsy and to start treatment with mefloquine. In the brain biopsy, histopathological features of demyelination were described and the polymerase chain reaction (PCR) identified JCV, confirming the diagnosis of PML. Treatment with mefloquine (250 mg/week) and dexamethasone (4 mg/day) was started and maintained for 6 months. A year later there was an almost complete resolution of the MRI lesions and the patient achieved a stable clinical state with persisting motor impairment and severe epilepsy. The patient is alive 38 months after diagnosis of PML, which is the longest known survival to date.
authors.affiliation: Department of Translational Research, MD Anderson Cancer Center Madrid, Madrid, Spain.
authors.forename: Heidys
authors.initials: H
authors.lastname: Garrote
authors.affiliation: Department of Hematology, MD Anderson Cancer Center Madrid, Madrid, Spain.
authors.forename: Adolfo
authors.initials: A
authors.lastname: de la Fuente
authors.affiliation: Department of Hematology, MD Anderson Cancer Center Madrid, Madrid, Spain.
authors.forename: Raquel
authors.initials: R
authors.lastname: Oña
authors.affiliation: Department of Radiology, MD Anderson Cancer Center Madrid, Madrid, Spain.
authors.forename: Inmaculada
authors.initials: I
authors.lastname: Rodríguez
authors.affiliation: Department of Virology, Centro Nacional de Microbiología, Instituto de Salud Carlos III, Madrid, Spain ; Centro de Investigación Biomédica en Red de Epidemiología y Salud Pública (CIBERESP), Madrid, Spain.
authors.forename: Juan E
authors.initials: JE
authors.lastname: Echevarría
authors.affiliation: Department of Neurology, MD Anderson Cancer Center Madrid, Madrid, Spain.
authors.forename: Juan M
authors.initials: JM
authors.lastname: Sepúlveda
authors.affiliation: Department of Translational Research, MD Anderson Cancer Center Madrid, Madrid, Spain ; Department of Pathology, MD Anderson Cancer Center, Madrid, Spain.
authors.forename: Juan F
authors.initials: JF
authors.lastname: García
keywords: Demyelization; Immune system suppression; John Cunningham virus; Progressive multifocal leukoencephalopathy