中华内科杂志
中華內科雜誌
중화내과잡지
CHINESE JOURNAL OF INTERNAL MEDICINE
2011年
5期
404-407
,共4页
翟勇平%宋萍%李锋%刘海宁%于亚平%周晓钢%史平%安志明%周晓%张春妮
翟勇平%宋萍%李鋒%劉海寧%于亞平%週曉鋼%史平%安誌明%週曉%張春妮
적용평%송평%리봉%류해저%우아평%주효강%사평%안지명%주효%장춘니
淀粉样变性%免疫球蛋白轻链%免疫组织化学%免疫固定电泳
澱粉樣變性%免疫毬蛋白輕鏈%免疫組織化學%免疫固定電泳
정분양변성%면역구단백경련%면역조직화학%면역고정전영
Amyloid degeneration%Immunoglobulin light chains%Immunohistochemistry%Immunofixation electrophoresis
目的 探讨血清游离轻链(sFLC)检测在原发性系统性(AL)淀粉样变性诊断和治疗中的意义.方法 收集我院2005年10月-2010年5月收治的25例AL淀粉样变性患者,男18例,女7例,平均年龄54(47～77)岁.采用免疫比浊法测定sFLC,以sFLC κ/λ比值判断单克隆轻链的类型,并与血清免疫固定电泳(IFE)、免疫组化方法比较敏感性.其中9例进行了治疗,观察治疗前后sFLC变化.结果 25例AL淀粉样变性患者中κ型2例,λ型23例.骨髓平均浆细胞比例3.5%(0～15%).19例(76%)患者的sFLC数值和κ/λ比值均异常,IFE阳性者为17例(68%),sFLC敏感性与IFE相当(P=0.727).21例(84%)患者肾活检和(或)肠黏膜活检病理组织免疫组化检测阳性.sFLC联合IFE阳性率88%,免疫组化联合sFLC或IFE阳性率均为96%.治疗后4例血液学缓解的患者器官功能均改善,5例未获缓解者病情稳定或进展.结论 sFLC测定是一种敏感的M蛋白定性定量方法,初步显示治疗后sFLC明显下降和(或)κ/λ比值恢复正常者,器官功能改善比例高,sFLC是AL淀粉样变性诊断的重要指标,并对疗效判定可能有帮助.
目的 探討血清遊離輕鏈(sFLC)檢測在原髮性繫統性(AL)澱粉樣變性診斷和治療中的意義.方法 收集我院2005年10月-2010年5月收治的25例AL澱粉樣變性患者,男18例,女7例,平均年齡54(47～77)歲.採用免疫比濁法測定sFLC,以sFLC κ/λ比值判斷單剋隆輕鏈的類型,併與血清免疫固定電泳(IFE)、免疫組化方法比較敏感性.其中9例進行瞭治療,觀察治療前後sFLC變化.結果 25例AL澱粉樣變性患者中κ型2例,λ型23例.骨髓平均漿細胞比例3.5%(0～15%).19例(76%)患者的sFLC數值和κ/λ比值均異常,IFE暘性者為17例(68%),sFLC敏感性與IFE相噹(P=0.727).21例(84%)患者腎活檢和(或)腸黏膜活檢病理組織免疫組化檢測暘性.sFLC聯閤IFE暘性率88%,免疫組化聯閤sFLC或IFE暘性率均為96%.治療後4例血液學緩解的患者器官功能均改善,5例未穫緩解者病情穩定或進展.結論 sFLC測定是一種敏感的M蛋白定性定量方法,初步顯示治療後sFLC明顯下降和(或)κ/λ比值恢複正常者,器官功能改善比例高,sFLC是AL澱粉樣變性診斷的重要指標,併對療效判定可能有幫助.
목적 탐토혈청유리경련(sFLC)검측재원발성계통성(AL)정분양변성진단화치료중적의의.방법 수집아원2005년10월-2010년5월수치적25례AL정분양변성환자,남18례,녀7례,평균년령54(47～77)세.채용면역비탁법측정sFLC,이sFLC κ/λ비치판단단극륭경련적류형,병여혈청면역고정전영(IFE)、면역조화방법비교민감성.기중9례진행료치료,관찰치료전후sFLC변화.결과 25례AL정분양변성환자중κ형2례,λ형23례.골수평균장세포비례3.5%(0～15%).19례(76%)환자적sFLC수치화κ/λ비치균이상,IFE양성자위17례(68%),sFLC민감성여IFE상당(P=0.727).21례(84%)환자신활검화(혹)장점막활검병리조직면역조화검측양성.sFLC연합IFE양성솔88%,면역조화연합sFLC혹IFE양성솔균위96%.치료후4례혈액학완해적환자기관공능균개선,5례미획완해자병정은정혹진전.결론 sFLC측정시일충민감적M단백정성정량방법,초보현시치료후sFLC명현하강화(혹)κ/λ비치회복정상자,기관공능개선비례고,sFLC시AL정분양변성진단적중요지표,병대료효판정가능유방조.
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.
