Simultaneous progressive multifocal leukoencephalopathy, Epstein-Barr virus (EBV) latent infection and cerebral parenchymal infiltration during chronic lymphocytic leukemia.
We report a non-HIV patient who had B chronic lymphocytic leukemia (CLL) with progressive multifocal leukoencephalopathy (PML) and diffuse cerebral leukemic parenchymal infiltration in the presence of JC virus and Epstein-Barr virus (EBV) cerebral co-infection. Multiple subcortical hypodensities lining the cortico-subcortical junction were present within the white matter on computerized tomography (CT) scan, with large areas of high signal intensity on T2-weighted sequences on magnetic resonance imaging (MRI). JCV DNA was identified in peripheral blood nuclear cells and cerebrospinal fluid polymerase chain reaction (PCR) DNA/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. Leukemic B cells with the same phenotype as leukemic blood cells were disseminated in the demyelinated areas. They were labeled by anti-latent membrane protein and by BamHl W EBV probe after ISH. Adhesion and activation molecules were positive for CD23. Autopsy showed diffuse visceral leukemic infiltration without acutization. EBV-transformed B lymphocytes would favour JCV penetration and/or intracerebral reactivation of previously latent JCV infection with further development of simultaneous PML and cerebral CLL infiltration in an immunosuppressed patient.
Farge
D
D
Department of Internal Medicine, Saint Louis Hospital, Paris VII University, France.
Hervé
R
R
Mikol
J
J
Sauvaget
F
F
Ingrand
D
D
Singer
B
B
Ferchal
F
F
Aupérin
I
I
Gray
F
F
Sudaka
A
A
D001402:B-Lymphocytes / Q000382:microbiology; D001921:Brain / Q000473:pathology*; D002472:Cell Transformation, Viral; D006566:Herpesviridae Infections / Q000150:complications* / Q000382:microbiology; D004854:Herpesvirus 4, Human / Q000502:physiology; D006801:Humans; D007577:JC Virus; D015451:Leukemia, Lymphocytic, Chronic, B-Cell / Q000150:complications* / Q000473:pathology; D017254:Leukemic Infiltration; D007968:Leukoencephalopathy, Progressive Multifocal / Q000150:complications*; D008213:Lymphocyte Activation; D008297:Male; D008875:Middle Aged; D030361:Papillomavirus Infections / Q000150:complications; D014412:Tumor Virus Infections / Q000150:complications* / Q000382:microbiology; D017735:Virus Latency