Central nervous system involvement in CLL: an international retrospective study by ERIC, the European Research Initiative on CLL
Year: 2025
Type: article
Study design: Observational Study
Abstract: ABSTRACT: Central nervous system involvement (CNSi) of chronic lymphocytic leukemia (CLL) is a rare condition with no consensus on diagnostic criteria and limited evidence for management and outcome. Here, we report an international, multicenter, retrospective study conducted by the European Research Initiative on CLL. The study defined CNSi of CLL by the following: (1) detection of CLL cells in the cerebrospinal fluid or confirmation of CLL infiltration of the CNS based on a tissue biopsy, (2) clinical or radiographic evidence of neurologic disease, and (3) the absence of other explanations for the neurologic findings. A total of 48 patients from 26 centers in 15 countries met all 3 diagnostic criteria of CLL-CNSi. Median age at diagnosis of CNSi was 64 years. Most patients were males (73%), had Binet stage A at CLL diagnosis (61%), and had untreated CLL at the time of CNSi (63%). Motor impairment was the most common symptom (38%) followed by visual impairment (32%). Of 47 patients who received treatment for CNSi, half (51%) received targeted agents, most often a Bruton tyrosine kinase inhibitor (BTKi), and 34% received chemoimmunotherapy. Initial treatment was highly effective, leading to a reduction (83%) or complete resolution (71%) of neurologic symptoms and imaging findings in most patients. The estimated 5-year overall survival (OS) from the CNSi diagnosis was 77.1%. The 5-year time to next treatment or death was 94% for patients treated with BTKi compared with 64% for those treated with CIT. Treatment-sensitive disease, represented by attainment of CNS complete response after initial therapy, was associated with longer OS.
Source: Blood Advances
Authors: Thomas Chatzikonstantinou, Mariia Mikhaleva, Christian Brieghel, Christina Papangelou, Eva Minga, Celso Arrais-Rodrigues, Sotiria Besikli-Dimou, Amalia Cerutti, Michael Doubek, Lisbeth Enggaard, Blanca Espinet, Blanca Ferrer-Lores, Jose Antonio Garcia Vela, Massimo Gentile, Eva Gimeno Vázquez, Odit Gutwein, Yair Herishanu, Paula Jablonowska-Babij, Ozren Jaksic, Par Josefsson, Elzbieta Kalicińska, Maria Kislova, Enrico Lista, Ioannis Kotsianidis, Juan Marquet, Enrica Antonia Martino, Ciaran McAuley, Maria José Mela Osorio, Riccardo Moia, Claudia Mosquera, Eugene Nikitin, Miguel Arturo Pavlovsky, Verena Pfister, Emine Merve Savaş, Lydia Scarfò, Matjaz Sever, Lev Shvidel, Martin Simkovic, Niki Stavroyianni, Persefoni Talimtzi, Antigoni Tranidou, Andrea Visentin, Georgios Vrachiolias, Ewa Wasik-Szczepanek, Tomasz Wróbel, Münci Yağcı, Alessandro Cellini, Anastasia Chatzidimitriou, Gianluca Gaidano, Mary Ann Anderson, Matthew S. Davids, Jennifer R. Brown, Carsten U. Niemann, Kostas Stamatopoulos, Paolo Ghia, Inhye E. Ahn, Maya Koren-Michowitz
Institutions: Centre for Research and Technology Hellas, Dana-Farber Cancer Institute, Copenhagen University Hospital, Rigshospitalet, Universidade Federal de São Paulo, G. Papanikolaou General Hospital, Central European Institute of Technology, Masaryk University, Central European Institute of Technology – Masaryk University, University Hospital Brno, Hospital Del Mar, Municipal Institute for Medical Research, Hospital del Mar Research Institute, Hospital Clínico Universitario de Valencia, Hospital Universitario Puerta de Hierro Majadahonda, Azienda Ospedaliera di Cosenza, University of Calabria, Tel Aviv University, Tel Aviv Sourasky Medical Center, University of Wrocław, Wroclaw Medical University, University of Zagreb, University Hospital Dubrava, Botkin Hospital, Ospedale Santa Chiara, Provincia Autonoma di Trento, Democritus University of Thrace, University Hospital of Alexandroupolis, Hospital Universitario Ramón y Cajal, Ospedale Annunziata di Cosenza, The Royal Melbourne Hospital, Peter MacCallum Cancer Centre, Fundación para la Investigación, Docencia y Prevención del Cáncer, Università degli Studi del Piemonte Orientale “Amedeo Avogadro”, Gazi University, Vita-Salute San Raffaele University, IRCCS Ospedale San Raffaele, University of Ljubljana, Ljubljana University Medical Centre, Kaplan Medical Center, Charles University, University Hospital Hradec Králové, University of Padua, Medical University of Lublin, The University of Melbourne, Walter and Eliza Hall Institute of Medical Research
Language: English
Subfield: Genetics
Field: Medicine
Domain: Health Sciences
Open Access status: gold
Estimated APC paid: $3,800
Award: G2406870047
OpenAlex ID: W4415526615