Author(s)

T. M. Sandouno^1*, S. Chebrek², C. Tchonko², G. Pichancourt³, L. Giovannetti², H. Zerazhi², Romain Appay⁴, H. Lepidi⁴, H. Bachir¹, S. Hamaz¹, H. B. Alaoui¹, K. Serraj¹, B. Slama²

¹Internal Medicine Department, Mohammed VI University Hospital Center, Mohammed I University, Oujda, Morocco.
²Onco-hematology department, Henri Duffaut Hospital Center, Avignon, France.
³Infectiology Departement, Henri Duffaut Hospital Center, Avignon, France.
⁴Department of Pathological Anatomy and Neuropathology, Timone University Hospital Center, Marseille, France.

Background: Central neurological involvement is the most frequent extra hematological manifestation of chronic lymphocytic leukemia; it is multifactorial and rarely due to a cerebral localization of the disease. We report a case of cerebral localization of chronic lymphoid leukemia whose clinical and radiological aspects were very suggestive of progressive multifocal leukoencephalopathy. Case Presentation: A 65-year-old patient who was HIV-negative (human immunodeficiency virus), had consulted for bilateral axillary, cervical and inguinal lymphadenopathy associated with major asthenia and hyper lymphocytosis (lymphocyte count was 11 giga/l). Chronic lymphocyticleukemia with TP53 mutation was diagnosed and treatment with Ibrutinib 420 mg/day was initiated. After 2 months of treatment, the evolution was marked by the onset of neurological disorders whose clinical-radiological presentation and temporal evolution had led to the diagnosis of progressive multifocal leukoencephalopathy. In the absence of virological evidence in the cerebrospinal fluid analysis, a stereotactic biopsy of the brain lesions had been performed, making it possible to formally rule out this infectious hypothesis and to demonstrate cerebral invasion by tumour cells. Immuno-chemotherapy combining Rituximab-Cyclophosphamide-Doxorubicin-Vincristine-Prednisone-Ibrutinib (RCHOP-Ibrutinib) with intrathecal chemotherapy resulted in a very good clinical-radiological response. Conclusion: The appearance of neurological manifestations in the context of chronic lymphocytic leukemia must systematically lead to a search for a cerebral localization of the disease. In the absence of virological evidence in the cerebrospinal fluid, any suspicion of progressive multifocal leukoencephalopathy in this context should lead to the histological study of brain lesions.

Progressive Multifocal Leukoencephalopathy, Chronic Lymphocytic Leukemia, John Cunningham Virus, cerebral Localization, Ibrutinib

Sandouno, T. , Chebrek, S. , Tchonko, C. , Pichancourt, G. , Giovannetti, L. , Zerazhi, H. , Appay, R. , Lepidi, H. , Bachir, H. , Hamaz, S. , Alaoui, H. , Serraj, K. and Slama, B. (2020) Cerebral Localization of Chronic Lymphocytic Leukemia Simulating Progressive Multifocal Leukoencephalopathy: The Lessons from a Case. Case Reports in Clinical Medicine, 9, 96-106. doi: 10.4236/crcm.2020.94015.