D. Farge et al., SIMULTANEOUS PROGRESSIVE MULTIFOCAL LEUKOENCEPHALOPATHY, EPSTEIN-BARR-VIRUS (EBV) LATENT INFECTION AND CEREBRAL PARENCHYMAL INFILTRATION DURING CHRONIC LYMPHOCYTIC-LEUKEMIA, Leukemia, 8(2), 1994, pp. 318-321
We report a non-HIV patient who had B chronic lymphocytic leukemia (CL
L) with progressive multifocal leukoencephalopathy (PML) and diffuse c
erebral leukemic parenchymal infiltration in the presence of JC virus
and Epstein-Barr virus (EBV) cerebral co infection. Multiple subcortic
al hypodensities lining the cortico-subcortical junction were present
within the white matter on computerized tomography (CT) scan, with lar
ge areas of high signal intensity on T2-weighted sequences on magnetic
resonance imaging (MRI). JCV DNA was identified in peripheral blood n
uclear cells and cerebrospinal fluid polymerase chain reaction (PCR) D
NA/DNA hybridization plus Southern blot analysis. Frontal stereotactic
biopsy confirmed the diagnosis of PML by immunocytochemistry, in situ
hybridization (ISH) with JC Enzo probe and electron microscopy. Leuke
mic B cells with the same phenotype as leukemic blood cells were disse
minated in the demyelinated areas. They were labeled by anti-latent me
mbrane protein and by BamHI W EBV probe after ISH. Adhesion and activa
tion molecules were positive for CD23. Autopsy showed diffuse visceral
leukemic infiltration without acutization. EBV-transformed B lymphocy
tes would favour JCV penetration and/or intracerebral reactivation of
previously latent JCV infection with further development of simultaneo
us PML and cerebral CLL infiltration in an immuno-suppressed patient.