Richter's transformation in patients with chronic lymphocytic leukaemia: a Nationwide Epidemiological Study.

Autor: Ben-Dali Y; Department of Haematology, Rigshospitalet, Copenhagen University Hospital, Copenhagen, Denmark., Hleuhel MH; Department of Haematology, Rigshospitalet, Copenhagen University Hospital, Copenhagen, Denmark., da Cunha-Bang C; Department of Haematology, Rigshospitalet, Copenhagen University Hospital, Copenhagen, Denmark.; Department of Haematology, Roskilde University, Roskilde, Denmark., Brieghel C; Department of Haematology, Rigshospitalet, Copenhagen University Hospital, Copenhagen, Denmark., Poulsen CB; Department of Haematology, Roskilde University, Roskilde, Denmark., Clasen-Linde E; Department of Pathology, Rigshospitalet, Copenhagen University Hospital, Copenhagen, Denmark., Bentzen HHN; Department of Haematology, Aarhus University Hospital, Aarhus, Denmark., Frederiksen H; Department of Haematology, Odense University Hospital, Odense, Denmark., Christiansen I; Department of Haematology, Aalborg University Hospital, Aalborg, Denmark., Nielsen LH; Department of Haematology, Sydvestjysk Sygehus, Esbjerg, Denmark., Enggaard L; Department of Haematology, Herlev and Gentofte Hospital, Hellerup, Denmark., Helleberg M; Department of Infectious Diseases, Rigshospitalet, Copenhagen University Hospital, Copenhagen, Denmark., Clausen M; Department of Haematology, Sygehus Lillebaelt, Vejle, Denmark., Frederiksen M; Department of Haematology, Sygehus Soenderjylland, Sønderborg, Denmark., Pedersen RS; Department of Haematology, Hospitalsenheden Vest, Herning, Denmark., Niemann CU; Department of Haematology, Rigshospitalet, Copenhagen University Hospital, Copenhagen, Denmark., Andersen MA; Department of Haematology, Rigshospitalet, Copenhagen University Hospital, Copenhagen, Denmark.
Jazyk: angličtina
Zdroj: Leukemia & lymphoma [Leuk Lymphoma] 2020 Jun; Vol. 61 (6), pp. 1435-1444. Date of Electronic Publication: 2020 Feb 07.
DOI: 10.1080/10428194.2020.1719092
Abstrakt: Richter's transformation (RT) refers to the development of an aggressive lymphoma in patients with chronic lymphocytic leukemia (CLL). Approximately, 2-10% of patients with CLL develop RT, most often as diffuse large B-cell lymphoma. To assess the incidence of RT, we examined risk factors for RT and death among patients with RT in a nationwide CLL cohort (from 2008 to 2016). Among 3772 patients, 113 had biopsy-proven RT. With a median follow-up of 4.3 years, the 5-year cumulative incidence of RT was 2.8%. Advanced Binet stage (B/C) ( p <.001), unmutated IGHV ( p <.001), and del(17p) ( p <.001) were independently associated with risk of developing RT. Half of the patients with RT (49%) were treatment-naïve prior to transformation and demonstrated longer survival after RT compared to patients previously treated for CLL (6.1 vs. 2.8 years, p =.03). Whether this finding could be explained by a higher proportion of clonally unrelated RT among treatment-naïve patients, remain to be addressed.
Databáze: MEDLINE
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