Title :

Transformed waldenstrom macroglobulinaemia: clinical presentation and outcome. A multi-institutional retrospective study of 77 cases from the french innovative leukemia organization (filo)

Author(s) :

Durot, Eric [Auteur]
Tomowiak, Cécile [Auteur]
Michallet, Anne-Sophie [Auteur]
Dupuis, Jehan [Auteur]
Hivert, Benedicte [Auteur]
Lepretre, Stéphane [Auteur]
Toussaint, Elise [Auteur]
Godet, Sophie [Auteur]
Merabet, Fatiha [Auteur]
Van Den Neste, Eric [Auteur]
Ivanoff, Sarah [Auteur]
Roussel, Xavier [Auteur]
Zini, Jean-Marc [Auteur]
Regny, Caroline [Auteur]
Lemal, Richard [Auteur]
Sutton, Laurent [Auteur]
Perrot, Aurore [Auteur]
Le Du, Katell [Auteur]
Kanagaratnam, Lukshe [Auteur]
Morel, Pierre [Auteur]
Evaluation des technologies de santé et des pratiques médicales - ULR 2694 [METRICS]
Evaluation des technologies de santé et des pratiques médicales - ULR 2694 [METRICS]
Leblond, Véronique [Auteur]
Delmer, Alain [Auteur]

Journal title :

British journal of haematology

Abbreviated title :

Br. J. Haematol.

Volume number :

179

Pages :

439-448

Publication date :

2017-11-01

ISSN :

0007-1048

English keyword(s) :

Waldenstrom macroglobulinaemia
diffuse large B-cell lymphoma
histological transformation
extranodal involvement
chemoimmunotherapy

HAL domain(s) :

Sciences du Vivant [q-bio]

English abstract : [en]

Histological transformation (HT) to diffuse large B-cell lymphoma (DLBCL) is a rare and poorly reported complication of Waldenström macroglobulinaemia (WM). We performed a retrospective study of 77 WM patients with ...
Show more >Histological transformation (HT) to diffuse large B-cell lymphoma (DLBCL) is a rare and poorly reported complication of Waldenström macroglobulinaemia (WM). We performed a retrospective study of 77 WM patients with biopsy-proven transformation to DLBCL. The median time from WM diagnosis to HT was 4·6 years and 16 patients (21%) had never been treated for WM. At HT, extranodal sites were observed in 91% of patients with a rather high incidence of central nervous system, cutaneous or testicular involvement. Fluorodeoxyglucose-positron emission tomography was performed in half of the patients and the median maximum standardized uptake value was 15 for transformed disease. More than 80% of cases with available data for assessment by the Hans' algorithm harboured a non-germinal centre B-cell phenotype. First-line treatment for transformation consisted of R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine, prednisone)-like regimen in 85% of patients. The overall response rate after first-line treatment was 61% and the median overall survival was only 16 months for the entire cohort. Time to transformation above 5 years (P = 0·0004) and elevated LDH (P = 0·02) were associated with worse outcome. Based on these findings, HT should be considered and lead to a biopsy in WM patients presenting with extranodal involvement, elevated LDH and constitutional symptoms. The optimal therapeutic approaches remain to be defined.Show less >

Language :

Anglais

Audience :

Internationale

Popular science :

Non

Administrative institution(s) :

CHU Lille
Université de Lille

Collections :

• METRICS : Evaluation des technologies de santé et des pratiques médicales - ULR 2694

Submission date :

2019-12-09T18:17:23Z