Background Light-chain amyloidosis (AL) and multiple myeloma (MM) may coexist in some patients and, although they share some cytogenetic abnormalities, they usually present with different clinical phenotypes. Translocation (11;14) is the most common cytogenetic abnormality in AL, but the prevalence and clinical implication of t(11;14) in patients with AL, with or without coexistent MM, remains unclear.
Methods A total of 119 consecutive newly diagnosed AL patients with available fluorescence in situ hybridization (FISH) data were retrospectively included and classified as primary AL alone (pAL-alone) or AL with coexistent MM (AL-MM). Clinical characteristics, FISH profiles, and hematologic and survival outcomes were analyzed.
Results There were 53 patients in the pAL-alone group and 66 in the AL-MM group. The prevalence of t(11;14) was significantly higher in the pAL-alone group than the AL-MM group (49.1% vs. 26.2%, P=0.012). A significantly higher proportion of the pAL-alone group achieved hematologic response compared with the AL-MM group (60.4% vs. 39.4%, P=0.023). Patients with AL-MM experienced significantly shorter hematologic event-free survival (hemEFS) than those with pAL-alone (median, 4.8 months vs. 44.3 months, P<0.001), as well as significantly shorter overall survival (OS; median, 15.2 months vs. not reached, P<0.001). When stratified by the presence or absence of coexistent MM and t(11;14), AL-MM patients with t(11;14) had the worst hemEFS (median, 3.8 months, P<0.001) and OS (median, 5.4 months, P=0.001).
Conclusions Patients with pAL-alone had a higher prevalence of t(11;14) than those with AL-MM. The AL-MM group had poorer outcomes, despite the availability of proteasome inhibitor treatment, with AL-MM patients with t(11;14) showing the worst outcomes. Better diagnostic and treatment approaches are warranted for this population.
DownLoad: