
系统性轻链型淀粉样变性诊断和治疗指南(2021年修订)
血液学评估需根据患者基线血清游离轻链差值(dFLC)水平选择合适的标准,dFLC>50 mg/L的患者所有血液学反应均可评估,dFLC水平在20~50 mg/L的患者可评估完全缓解(CR)和部分缓解(PR),dFLC<20 mg/L的患者目前仍无法评估血液学疗效。
AL amyloidosis patients with low amyloidogenic free light …
2017年8月3日 · The difference between involved minus uninvolved serum free light chains (dFLC) has been established as an invaluable hematologic parameter in systemic amyloid light chain (AL) amyloidosis. However, patients with an initial dFLC level <50 mg/L are currently deemed not evaluable for response to therapy.
Systemic Light Chain Amyloidosis | New England Journal of Medicine
2024年6月26日 · Immunoglobulin light chain (AL) amyloidosis is a rare disease that occurs when a plasma cell disorder produces monoclonal light chains, which misfold and are deposited as...
梅奥Gertz:轻链型淀粉样变性的诊断、预后和治疗(2022更新)
2022年4月18日 · 对临床综合征符合AL淀粉样变性的患者应进行筛查,包括血清免疫固定电泳、尿免疫固定电泳和Ig游离轻链 (FLC)检测。 对于淀粉样变性,24小时尿液免疫固定至关重要。 高频率的λ轻链蛋白血症是AL淀粉样变性的标志。 如果血清和尿液免疫固定结果为阴性,Ig FLC (κ:λ)比值正常 (0.26-1.65),则AL淀粉样变性不太可能,不应进行进一步评价,除非临床怀疑指数非常高。 评价疑似淀粉样变性患者的策略见图1。 免疫球蛋白正常不能排除局限性或家族性淀粉样变性。
Management of AL amyloidosis in 2020
2020年12月3日 · The κ-free light chain (FLC) concentration was 206 mg/L (ratio [FLCR], 10.3, and differential FLC [dFLC], 186 mg/L); bone marrow plasma cell (PC) infiltrate was 12% without chromosomal abnormalities; blood count, calcium, and liver function test results were normal; estimated glomerular filtration rate (eGFR) was 48 mL/min; proteinuria was 2.8 ...
Difference in Involved and Uninvolved Free Light Chain (dFLC) of …
2019年11月13日 · Achieving dFLC <1mg/dL early post ASCT is significantly associated with renal response. Renal response is independent of baseline proteinuria and BM plasma cells or MRD status post ASCT. Our study supports that pathologic entity in organ damage is not the plasma cells but rather light chains.
血清游离轻链(dFLC) - CHINAGUT
① 受累的少量和未受累血清游离轻链(dFLC)之间的差别,已作为全身性轻链淀粉样变性(AL)中不可评价的血液学参数;② 回顾性分析2002年至2016年本中心新诊断的783例AL患者,dFLC50的患者,更少浆细胞增多症,但所有分析的染色体异常均无差异,心脏受累少见 ...
Clinical presentation and outcomes in light chain amyloidosis ... - PubMed
Hematologic response criteria in light chain (AL) amyloidosis require the difference in involved and uninvolved free light chains (dFLC) to be at least 5 mg/dl. We describe the clinical presentation and outcomes of newly diagnosed amyloidosis patients …
Early dFLC response by C1D7 predicts complete hematologic
2024年11月12日 · We propose a new concept of “the rapid dFLC response” in AL amyloidosis, based on treatment with daratumumab, bortezomib, and dexamethasone. Rapid dFLC response in C1D7 (67.0% change, sensitivity 87.5%, specificity 81.3%) can …
血清游离轻链检测在轻链型淀粉样变性检测中的必要性
2022年4月25日 · ① 部分缓解的标准:血清游离轻链差值(dFLC)>50 mg/L的患者:dFLC下降>50% ② dFLC在20~50 mg/L的患者:dFLC<10 mg/L. ③ 非常好的部分缓解的标准:dFLC下降至<40 mg/L. ④ 完全缓解的标准:血液/尿液免疫固定电泳阴性,并且血清游离轻链水平和比值正常。 ⑤ 严格意义的完全缓解的标准:符合完全缓解,并且血清游离轻链比值(iFLC) ≤20 mg/L和dFLC≤10 mg/L。 因此,血清游离轻链检测在疗效评估中有着决定性的作用。 3、对治疗的指 …