Clinical presentation and prognosis of immunoglobulin light-chain amyloidosis with high percentage of bone marrow plasma cells. (June 2019)

• Record Type: Journal Article
• Authors: Zhang, Cong-li; Qiu, Yu; Shen, Kai-ni; Miao, Hui-lei; Feng, Jun; Cao, Xin-xin; Zhang, Lu; Jiang, Xian-yong; Zhou, Dao-bin; Li, Jian
• Is Part Of: Leukemia research. Volume 81(2019)
• Page Start: 19
• Page End: 24
• Publication Date: 2019-06
• Subjects: AL amyloidosis immunoglobulin light-chain amyloidosis -- BMPCs bone marrow plasma cells -- dFLC difference between the involved and uninvolved serum free light chain -- ASCT autologous stem cell transplantation; Light-chain amyloidosis -- Bone marrow plasma cells -- Outcome; Leukemia -- Periodicals; Leukemia -- Periodicals; Leucémie -- Périodiques; Leukemia; Periodicals; Electronic journals; Electronic journals; 616.9941905
• DOI: 10.1016/j.leukres.2019.04.002
• Languages: English
• ISSNs: 0145-2126

Abstract

Highlights: Early mortality after diagnosis among AL amyloidosis patients with ≥10% BMPCs was 21.4%. BMPCs ≥10% was associated with shorter PFS and OS among AL amyloidosis patients. Bortezomib or lenalidomide-based therapy might not overcome the negative prognostic impact of high BMPCs. Abstract: Objective: To summarize the clinical features and outcomes in Chinese patients with immunoglobulin light-chain (AL) amyloidosis with ≥10% bone marrow plasma cells (BMPCs). Methods: We retrospectively compared the clinical features and outcomes between patients with ≥10% BMPCs (high-BMPC group; n = 56) and those with <10% BMPCs (low-BMPC group; n = 311). Results: Patients in the high-BMPC group had significantly higher levels of N-terminal pro-brain natriuretic peptide, significantly lower levels of 24 h urine protein, and significantly higher levels of difference between the involved and uninvolved serum free light chains (485.3 versus 121.1 mg/L, P < 0.001). Patients in the high-BMPC group had significantly higher early mortality within 3 months of diagnosis (21.4% versus 10.9%, P = 0.018). In a 3-month landmark analysis, median progression-free survival durations were 17.3 and 34.5 months (P = 0.01), and the median overall survival durations were 24.4 months and not reached in the high- and low-BMPC groups, respectively (P = 0.005). Conclusion: Patients with AL amyloidosis and ≥10% BMPCs have higher mortality within 3 months of diagnosis and poorer prognosis compared with patients with <10% BMPCs.