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Transformed Waldenström macroglobulinaemia: clinical presentation and outcome. A multi‐institutional retrospective study of 77 cases from the French Innovative Leukemia Organization ( FILO )
Author(s) -
Durot Eric,
Tomowiak Cécile,
Michallet AnneSophie,
Dupuis Jehan,
Hivert Bénédicte,
Leprêtre Stéphane,
Toussaint Elise,
Godet Sophie,
Merabet Fatiha,
Van Den Neste Eric,
Ivanoff Sarah,
Roussel Xavier,
Zini JeanMarc,
Regny Caroline,
Lemal Richard,
Sutton Laurent,
Perrot Aurore,
Le Dû Katell,
Kanagaratnam Lukshe,
Morel Pierre,
Leblond Véronique,
Delmer Alain
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.14881
Subject(s) - medicine , rituximab , retrospective cohort study , waldenstrom macroglobulinemia , cyclophosphamide , prednisone , lymphoma , diffuse large b cell lymphoma , biopsy , vincristine , gastroenterology , aggressive lymphoma , surgery , chemotherapy
Summary 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.