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    Association details:
    Biomarker:CXCR4 mutation
    Cancer:Waldenstrom Macroglobulinemia
    Drug:Rituxan (rituximab) (CD20 inhibitor) +
    Ninlaro (ixazomib) (Proteasome inhibitor)
    Direction:Sensitive
    Evidence:
    Evidence Level:
    Sensitive: C2 – Inclusion Criteria
    Source:
    clinicaltrials.gov
    Title:

    Trial of Ixazomib, Dexamethasone and Rituximab in Patients With Untreated Waldenstrom's Macroglobulinemia

    Excerpt:
    ...To evaluate the overall response rate of participants by MYD88 L265P and CXCR4-WHIM mutations in WM. ...
    Trial ID:
    NCT02400437
    Evidence Level:
    Sensitive: C3 – Early Trials
    Source:
    ASH 2019
    Title:

    CXCR4 Mutational Status Does Not Impact Outcomes in Patients with Waldenstrom Macroglobulinemia Treated with Proteasome Inhibitors

    Published date:
    11/06/2019
    Excerpt:
    The median PFS for patients without CXCR4 mutations was 3.6 years (95% CI 1.7-5.9 years) versus 6.5 years (95% CI 2.7-not reached) for patients with CXCR4 mutations, which was not statistically significantly different (HR 0.59, 95% CI 0.29-1.17; p=0.13). Frameshift CXCR4 mutations were associated with better PFS than nonsense CXCR4 mutations in a multivariate Cox regression model (HR 0.22, 95% CI 0.06-0.80; p=0.02). Patients with CXCR4 mutations had a 10-year SAFTI rate of 75% (95% CI 13-96%) versus 83% (95% CI 27-97%) in patients without CXCR4 mutations, which was not statistically significantly different (HR 3.01, 95% CI 0.17-52.6; p=0.45).
    Secondary therapy:
    dexamethasone
    DOI:
    https://doi.org/10.1182/blood-2019-122349