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轻链淀粉样变性流式免疫分型的临床应用及其对预后的意义

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发表于 2012-11-2 12:54:40 | 显示全部楼层 |阅读模式

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The clinical utility and prognostic value of multiparameter flow cytometry immunophenotyping in light-chain amyloidosis
*Bruno Paiva 等人

The clinical value of multiparameter flow cytometry (MFC) immunophenotyping in primary or light chain amyloidosis (AL) remains unknown. We studied 44 consecutive bone marrow samples from newly diagnosed patients with amyloidosis; 35 patients with AL and 9 with other forms of amyloidosis. Monoclonal plasma cells (PCs) were identifiable by MFC immunophenotyping in 34 of 35 (97%) patients with AL, whereas it was absent from all but 1 of the 9 (11%) patients with other forms of amyloidosis. Quantification of bone marrow plasma cells (BMPCs) by MFC immunophenotyping was a significant prognostic factor for overall survival (OS) (< 1% vs > 1% BMPC cutoff; 2-year OS rates of 90% vs 44%, P  .02). Moreover, detecting persistent normal PCs at diagnosis identifies a subgroup of patients with AL with prolonged OS (> 5% vs < 5% normal PC within all BMPC cutoff, 2-year rates of 88% vs 37%, P  .01). MFC immunophenotyping could be clinically useful for the demonstration of PC clonality in AL and for the prognostication of patients with AL. (Blood. 2011; 117(13):3613-3616)

轻链淀粉样变性介绍:Primary or light chain amyloidosis (AL) is the most frequent type of systemic amyloidosis and is characterized by the accumulation of monoclonal light chain fragments, leading to end-organ damage and short survival

骨髓浆细胞数量对预后的影响(见下图):

骨髓浆细胞数量对预后的影响

骨髓浆细胞数量对预后的影响

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