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

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  • Yasmin Ben-Dali, Rigshospitalet
  • ,
  • Mariam H. Hleuhel, Rigshospitalet
  • ,
  • Caspar da Cunha-Bang, Rigshospitalet, Roskilde University
  • ,
  • Christian Brieghel, Rigshospitalet
  • ,
  • Christian B. Poulsen, Department of Haematology, Roskilde University
  • ,
  • Erik Clasen-Linde, Rigshospitalet
  • ,
  • Hans H.N. Bentzen
  • Henrik Frederiksen
  • Ilse Christiansen
  • Linda H. Nielsen, Sydvestjysk Sygehus, Esbjerg
  • ,
  • Lisbeth Enggaard, Gentofte Hospital
  • ,
  • Marie Helleberg, Rigshospitalet
  • ,
  • Michael Clausen
  • Mikael Frederiksen, Sygehus Sønderjylland
  • ,
  • Robert S. Pedersen, Hospitalsenheden Vest
  • ,
  • Carsten U. Niemann, Rigshospitalet
  • ,
  • Michael A. Andersen, Rigshospitalet

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.

OriginalsprogEngelsk
TidsskriftLeukemia and Lymphoma
Vol/bind61
Nummer6
Sider (fra-til)1435-1444
Antal sider10
ISSN1042-8194
DOI
StatusUdgivet - jun. 2020

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