Central nervous system localisation of chronic lymphocytic leukaemia, description of two very distinct cases and a review of the literature

Abstract

Central nervous system (CNS) localisation of chronic lymphocytic leukaemia (CLL) can induce various neurological symptoms. Unfamiliarity with this manifestation causes diagnostic delay. We present two cases of leptomeningeal CLL. These cases and our literature review emphasise that CNS localisation of CLL should be considered in patients with any neurological symptom, irrespectively of the stage and systemic activity of CLL.Electronic supplementary materialThe online version of this article (10.1007/s00277-018-3329-2) contains supplementary material, which is available to authorized users.

Cerebral Localization of Chronic Lymphocytic Leukemia Simulating Progressive Multifocal Leukoencephalopathy: The Lessons from a Case

“…The non-specific nature of the symptoms justifies the diagnostic delay with an average delay of 5 months. The leptomeningeal localization was the most represented, confirmed most often by the cytological study and CSF flow cytometry [14] [15]. No correlation has been established between the stage of CLL and the occurrence of neurological complications; indeed neurological localization has been diagnosed in patients in the early stage of the disease or in completely asymptomatic patients with no systemic CLL activity [17].…”

“…To date, no study has been able to identify a risk factor for neurological progression of CLL, although soluble CD27 and the study of CD49d/CD82 expression have been proposed as biomarkers of risk [16] [18]. Overall, a favourable therapeutic response was obtained [14] [15].…”

Venetoclax with high‐dose methotrexate and rituximab seem effective and well‐tolerated in the treatment of central nervous system involvement of chronic lymphocytic leukemia: A case report

“…Chronic lymphocytic leukemia (CLL) with central nervous system (CNS) involvement is a rare and severe condition . There is currently no consensus regarding optimal management .…”

“…Chronic lymphocytic leukemia (CLL) with central nervous system (CNS) involvement is a rare and severe condition. 1,2 There is currently no consensus regarding optimal management. 3,4 Venetoclax, an oral BCL-2 inhibitor, has recently proven to be effective for refractory/relapsed CLL when administered with or without rituximab.…”

Central Nervous System Involvement by Small Lymphocytic Lymphoma after a Myxoma-Related Embolic Event

“…Infiltration of CLL/SLL cells outside lymphoid tissues, called extramedullary CLL (EM-CLL), is rare. Though a common and potentially devastating complication of aggressive lymphomas like Burkitt lymphoma (BL), fewer than 200 cases of central nervous system (CNS) involvement by CLL/SLL are reported in the literature [2]. Most commonly, it is described in patients with high counts of malignant lymphocytes in peripheral blood [3].…”