Light-chain Amyloidosis with Concomitant Symptomatic Myeloma (CRAB-SLiM Features): Clinical Characteristics, Cytogenetic Abnormalities, and Outcomes

Abstract

Abstract Patients with light chain (AL) amyloidosis and concomitant symptomatic multiple myeloma (MM) are known to have a worse prognosis, while the prognostic implication of cytogenetic abnormalities (CA) and optimal treatment schemes are not well-established. This study retrospectively evaluated the clinical characteristics, CA, and outcomes of 106 patients with AL amyloidosis and concomitant symptomatic MM (MM-AL) by comparing with patients with MM (n=658) or primary AL amyloidosis (pAL, n=151) alone. The prevalence of t(11;14) was significantly higher in the pAL-alone group than in MM-AL and MM-alone group (40.7 % vs. 25.7% vs. 16.6%, p<0.001), and the prevalence of del13q, gain1q21 and high-risk CA (HRCA) decrease in turn in MM-alone, MM-AL and pAL-alone group. The progression-free survival (PFS) and overall survival (OS) of MM-AL patients (median, 12.8 and 25.2 months) were significantly inferior to patients with MM-alone and pAL-alone. No significant difference in PFS and OS was found between MM-AL patients with and without HRCA. When stratified by the type of plasma cell disease and status of t(11;14), patients with MM-AL and t(11;14) presented the worst OS (median, 8.2 months, p<0.001). Regarding the management of MM-AL, extended cycles of induction therapy and the use of maintenance therapy contributed to a better prognosis.