Impact of MYD88L265P mutation status on histological transformation of Waldenström Macroglobulinemia

Saurabh Zanwar, Jithma P. Abeykoon, Eric Durot, Rebecca King, Gabriela E. Perez Burbano, Shaji Kumar, Morie A. Gertz, Anne Quinquenel, Alain Delmer, Wilson Gonsalves, Pascale Cornillet-Lefebvre, Rong He, Rahma Warsame, Francis K. Buadi, Anne J. Novak, Patricia T. Greipp, David Inwards, Thomas M. Habermann, Ivana Micallef, Ronald GoEli Muchtar, Taxiarchis Kourelis, Angela Dispenzieri, Martha Q. Lacy, David Dingli, Grzegorz Nowakowski, Carrie A. Thompson, Patrick Johnston, Gita Thanarajasingam, N. Nora Bennani, Thomas E. Witzig, Jose Villasboas, Nelson Leung, Yi Lin, Robert A. Kyle, S. Vincent Rajkumar, Stephen M. Ansell, Jennifer G. Le-Rademacher, Prashant Kapoor

Research output: Contribution to journalArticlepeer-review

4 Scopus citations

Abstract

Histological transformation in Waldenström macroglobulinemia (WM) is an uncommon complication, with limited data, particularly regarding the impact of MYD88L265P mutation on transformation. We examined risk factors and outcomes associated with transformation in WM, highlighting the role of MYD88L265P mutation. Patients with WM seen at Mayo Clinic, Rochester, USA and University Hospital of Reims, France, between 01/01/1996 and December 31, 2017 were included; 50 (4.3%) of 1147 patients transformed to a high-grade lymphoma, with median time-to-transformation of 4.5 (range 0-21) years in the transformed cohort. The MYD88L265P mutation status was known in 435/1147 (38%) patients (406 with non-transformed WM and 29 patients in transformed cohort). On multivariate analysis, MYD88WT status alone was an independent predictor of transformation (odds ratio, 7[95%CI: 2.1-23]; P =.003). Additionally, the MYD88WT status was independently associated with shorter time-to-transformation (HR 7.9 [95%CI: 2.3-27; P =.001]), with a 5-year transformation rate of 16% for MYD88WT vs 2.8% with MYD88L265P mutated patients. Patients with transformation demonstrated a significant increase in risk of death compared to patients who did not transform (HR 5.075; 95%CI: 3.8-6.8; P <.001). In conclusion, the MYD88WT status is an independent predictor of transformation and associated with a shorter time-to-transformation. Additionally, transformation conferred an inferior overall survival in patients with WM.

Original languageEnglish (US)
Pages (from-to)274-281
Number of pages8
JournalAmerican journal of hematology
Volume95
Issue number3
DOIs
StatePublished - Mar 1 2020

ASJC Scopus subject areas

  • Hematology

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