Halushka Marc K, Eng George, Collins A Bernard, Judge Daniel P, Semigran Marc J, Stone James R
Department of Pathology, Johns Hopkins University School of Medicine, Baltimore, MD, USA.
J Cardiovasc Transl Res. 2015 Jun;8(4):264-8. doi: 10.1007/s12265-015-9628-2. Epub 2015 Apr 30.
Accurate and rapid classification of cardiac amyloidosis is important for patient management. We have optimized the use of serum free light chain kappa and lambda values to differentiate immunoglobulin light chain amyloid (AL) amyloidosis from transthyretin amyloid and amyloid A using 85 cases of tissue-proven cardiac amyloidosis, in which there was direct classification of amyloidosis by mass spectrometry or immunofluorescence. The serum free light chain kappa/lambda ratios were non-overlapping for the three major groups: AL-lambda (0.01-0.41, n = 30), non-AL (0.52-2.7, n = 43), and AL-kappa (6.7-967, n = 12). A kappa/lambda ratio value between 0.5 and 5.0 had 100 % sensitivity and 100 % specificity for distinguishing AL amyloidosis from non-AL amyloidosis. This optimized range for serum light chain kappa/lambda ratio provides extremely robust classification of cardiac amyloidosis. Cases of cardiac amyloidosis in which the serum kappa/lambda free light chain ratio falls close to these new cutoff values may benefit most from direct amyloid subtyping.
准确快速地对心脏淀粉样变性进行分类对于患者管理至关重要。我们利用85例经组织证实的心脏淀粉样变性病例，优化了血清游离轻链κ和λ值的应用，以区分免疫球蛋白轻链淀粉样变性（AL）与转甲状腺素蛋白淀粉样变性和淀粉样蛋白A，其中通过质谱或免疫荧光对淀粉样变性进行了直接分类。血清游离轻链κ/λ比值在三个主要组中不重叠：AL-λ（0.01 - 0.41，n = 30）、非AL（0.52 - 2.7，n = 43）和AL-κ（6.7 - 967，n = 12）。κ/λ比值在0.5至5.0之间对区分AL淀粉样变性与非AL淀粉样变性具有100%的敏感性和100%的特异性。血清轻链κ/λ比值的这一优化范围为心脏淀粉样变性提供了极为可靠的分类。血清κ/λ游离轻链比值接近这些新临界值的心脏淀粉样变性病例可能最受益于直接淀粉样变性亚型分类。
