@article{3106456,
    title = "Evaluating ibrutinib in the treatment of symptomatic Waldenstrom’s macroglobulinemia",
    author = "Papanota, A.-M. and Ntanasis-Stathopoulos, I. and Kastritis, E. and Dimopoulos, M.A. and Gavriatopoulou, M.",
    journal = "Journal of blood medicine",
    year = "2019",
    volume = "10",
    pages = "291-300",
    publisher = "Dove Medical Press Ltd",
    doi = "10.2147/JBM.S183997",
    keywords = "acalabrutinib;  B lymphocyte receptor;  bendamustine;  bortezomib;  Bruton tyrosine kinase;  chemokine receptor CXCR4;  daratumumab;  ibrutinib;  immunoglobulin M;  lenalidomide;  myeloid differentiation factor 88;  pembrolizumab;  pevonedistat;  phospholipase C gamma2;  rituximab;  ulocuplumab;  zanubrutinib, atrial fibrillation;  blood clotting;  chronic lymphatic leukemia;  clinical practice;  continuous infusion;  disease course;  disease exacerbation;  drug indication;  drug mechanism;  drug response;  drug screening;  fatigue;  gastrointestinal disease;  human;  immunoglobulin deficiency;  overall survival;  patient care;  phase 3 clinical trial;  predictive value;  progression free survival;  rash;  Review;  somatic mutation;  treatment response;  upper respiratory tract infection;  Waldenstroem macroglobulinemia;  whole genome sequencing",
    abstract = "Waldenstrom’s macroglobulinemia (WM) is a rare lymphoplasmacytic lymphoma with indolent course and prolonged disease course. The first-in-class Bruton’s tyrosine kinase inhibitor, ibrutinib, has shown significant activity and a distinct adverse event profile among both newly diagnosed and relapsed/refractory WM patients. Interestingly, clinical responses to ibrutinib have been shown to be dependent on patients’ MYD88 and CXCR4 mutational status. The recent outcomes of the Phase III iNNOVATE trial showed that the combination of ibrutinib with rituximab resulted in a significantly prolonged progression-free survival compared with rituximab monotherapy, which provides a novel therapeutic option in the clinical practice especially for the rituximab-refractory WM patients. However, the need for continuous drug administration along with the unique toxicity manifestations may render the patient management challenging. Furthermore, our understanding of the underlying resistant mechanisms to ibrutinib is currently being evolved. © 2019 Papanota et al."
}