Abstract
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.
Highlights
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
Progressive multifocal leukoencephalopathy (PML) is a central nervous system infection caused by the John Cunningham Virus (JCV) and promoted by immunosuppression
Summary
Chronic Lymphocytic Leukemia (CLL) is the most common malignant hemopathy after the age of 60 It is characterized by a monoclonal proliferation of mature B lymphocytes infiltrating the bone marrow and the lymphoid organs, responsible for hyper lymphocytosis in the blood with or without tumor syndrome (lymphadenopathy, hepatosplenomegaly) [1]. Outside of these classical sanctuaries, extra-hematological tumor infiltration is a rare situation. A classic complication of HIV, PML is found in lymphoproliferative syndromes where it can be responsible for non-specific neurological manifestations with a radiological expression that is most often quite evocative [3]. We report a case of cerebral localization of CLL whose clinical-radiological and evolutionary aspects were very suggestive of progressive multifocal leukoencephalopathy
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