Transformed Waldenström macroglobulinaemia: clinical presentation and outcome. A multi-institutional retrospective study of 77 cases from the French Innovative Leukemia Organization (FILO).
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.
['Durot|Eric|E|0000-0003-3463-0089', 'Tomowiak|Cécile|C|', 'Michallet|Anne-Sophie|AS|', 'Dupuis|Jehan|J|', 'Hivert|Bénédicte|B|', 'Leprêtre|Stéphane|S|', 'Toussaint|Elise|E|', 'Godet|Sophie|S|', 'Merabet|Fatiha|F|', 'Van Den Neste|Eric|E|', 'Ivanoff|Sarah|S|', 'Roussel|Xavier|X|', 'Zini|Jean-Marc|JM|', 'Regny|Caroline|C|', 'Lemal|Richard|R|', 'Sutton|Laurent|L|', 'Perrot|Aurore|A|', 'Le Dû|Katell|K|', 'Kanagaratnam|Lukshe|L|', 'Morel|Pierre|P|', 'Leblond|Véronique|V|', 'Delmer|Alain|A|']
D000328:Adult D000368:Aged D000369:Aged, 80 and over D058846:Antibodies, Monoclonal, Murine-Derived D000971:Antineoplastic Combined Chemotherapy Protocols D014408:Biomarkers, Tumor D001706:Biopsy D002471:Cell Transformation, Neoplastic D003520:Cyclophosphamide D004317:Doxorubicin D005260:Female D019788:Fluorodeoxyglucose F18 D006801:Humans D007770:L-Lactate Dehydrogenase D016403:Lymphoma, Large B-Cell, Diffuse D008297:Male D008875:Middle Aged D049268:Positron-Emission Tomography D011241:Prednisone D011379:Prognosis D012189:Retrospective Studies D012307:Risk Factors D000069283:Rituximab D016019:Survival Analysis D014057:Tomography, X-Ray Computed D016896:Treatment Outcome D014750:Vincristine D008258:Waldenstrom Macroglobulinemia
Waldenström macroglobulinaemia histological transformation diffuse large B-cell lymphoma extranodal involvement chemoimmunotherapy