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Management of central nervous system involvement in chronic lymphocytic leukaemia: a retrospective cohort of 30 patients
Author(s) -
Wanquet Anne,
Birsen Rudy,
Bonnet Charlotte,
Boubaya Marouane,
Choquet Sylvain,
Dupuis Jehan,
Lepretre Stephane,
Re Daniel,
Fahri Jonathan,
Michallet AnneSophie,
Ysebaert Loïc,
Lemal Richard,
Lamy Thierry,
Delarue Richard,
Troussard Xavier,
Cymbalista Florence,
Levy Vincent,
Dietrich PierreYves,
Leblond Veronique,
AurranSchleinitz Therese
Publication year - 2017
Publication title -
british journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.907
H-Index - 186
eISSN - 1365-2141
pISSN - 0007-1048
DOI - 10.1111/bjh.14387
Subject(s) - medicine , fludarabine , retrospective cohort study , cohort , chronic lymphocytic leukemia , chemotherapy , ibrutinib , central nervous system , gastroenterology , oncology , cyclophosphamide , leukemia
Summary Central nervous system involvement ( CNS i) is a rare and poorly reported complication of chronic lymphocytic leukaemia ( CLL ). Establishing cause and effect between the CLL and the neurological symptoms remains challenging. We have analysed a retrospective cohort of 30 CLL patients with CNS i, documented by lymphocytic infiltration either by flow cytometry of the cerebrospinal fluid ( CSF ; n = 29) or CNS biopsy ( n = 1). Neurological symptoms were heterogeneous. At the time of CNS i, less than half of the patients had a progressive CLL and 20 had never been treated for CLL . Initial treatment with fludarabine‐based immuno‐chemotherapy, with or without intra‐ CSF therapy, led to durable response in eight out of nine untreated patients. In contrast, 50% patients receiving various prior treatments needed additional therapy within a median of 4 months (1–16). Ibrutinib led to complete response in 4/4 heavily pre‐treated patients. From CNS i, 5‐year overall survival was 72% and 48% for treatment‐naïve and previously treated patients respectively ( P = 0·06); 5‐year progression‐free survival ( PFS ) was 43% and 0% ( P = 0·125). 17p deletion was significantly associated with poor PFS ( P = 0·006). CNS i may be the only sign of progression of CLL and should be considered an initiation criterion of systemic treatment. Prognosis seemed to be related to CLL characteristics rather than to CNS i itself.