The clinical significance of serum free light chain in primary systemic amyloidosis
10.3760/cma.j.issn.0578-1426.2011.05.012
- VernacularTitle:血清游离轻链检测在原发性系统性淀粉样变性中的临床意义
- Author:
Yongping ZHAI
;
Ping SONG
;
Feng LI
;
Haining LIU
;
Yaping YU
;
Xiaogang ZHOU
;
Ping SHI
;
Zhiming AN
;
Xiao ZHOU
;
Chunni ZHANG
- Publication Type:Journal Article
- Keywords:
Amyloid degeneration;
Immunoglobulin light chains;
Immunohistochemistry;
Immunofixation electrophoresis
- From:
Chinese Journal of Internal Medicine
2011;50(5):404-407
- CountryChina
- Language:Chinese
-
Abstract:
Objective To evaluate the diagnostic and therapeutic significance of serum free light chain (sFLC) in primary systemic(AL) amyloidosis. Methods Twenty-five patients with AL amyloidosis,including 18 men and 7 women with a mean age of 54(47-77) years old, were enrolled from October, 2005to May, 2010. sFLC was measured by immunoturbidimetric assay. The type of monoclonal light chain was judged upon sFLC κ/λ and its sensibility was compared with serum immunofixation and immunohistochemical analysis. Four patients were treated with M (T)D (melphalan/thalidomideand, dexamethasone), one with VD (velcade and dexamethasone) and four with high-dose melphalan followed by autologous stem cell support. The changes of sFLC were serially determined before and after treatment. Results Among the 25 patients with AL amyloidosis, two were κ light chains of precursor protein and 23 were λ light chains. Mean plasma cell in bone marrow was 3.5% (0-15%). Nineteen (76%) patients had abnormal elevated sFLC and abnormal κ/λ ratios, and 17(68% ) patients with immunofixation positive. The sFLC test had similar sensitivity as serum immunofixation (P = 0. 727 ). Twenty-one (84%) patients were shown to have either κor λ immunoreactive amyloid deposits on biopsied tissues. The sFLC test combined with serum immunofixation allowed the M protein to be detected in 22 (88%) patients. The positive rates of immunohistochemical analysis combined with sFLC test and/or serum immunofixation were 96%. Four patients with hematologic response showed obvious improvement in visceral organ involvement, but illness of 5 patients without hematologic response kept stable or progressed. Conclusions sFLC test is a sensitive qualitative and quantitative method to detect M protein. Preliminary data show the patients with obvious sFLC level decrease and/or κ/λ recovery to normal may have a high percentage of improved organs function. sFLC is critical index in diagnosing AL amyloidosis, which might help efficacy assessment.
