Dispenzieri Angela, Gertz Morie A, Kyle Robert A, Lacy Martha Q, Burritt Mary F, Therneau Terry M, McConnell Joseph P, Litzow Mark R, Gastineau Dennis A, Tefferi Ayalew, Inwards David J, Micallef Ivana N, Ansell Stephen M, Porrata Luis F, Elliott Michelle A, Hogan William J, Rajkumar S Vincent, Fonseca Rafael, Greipp Philip R, Witzig Thomas E, Lust John A, Zeldenrust Steven R, Snow Denise S, Hayman Susan R, McGregor Christopher G A, Jaffe Allan S
Division of Hematology and Internal Medicine, Mayo Clinic, 200 First Street SW, Rochester, MN 55905, USA.
Blood. 2004 Sep 15;104(6):1881-7. doi: 10.1182/blood-2004-01-0390. Epub 2004 Mar 25.
Primary systemic amyloidosis (AL) is a fatal plasma cell disorder. Pilot data suggest survival is better in patients undergoing peripheral blood stem cell transplantation (PBSCT), but the selection process makes the apparent benefit suspect. We have reported that circulating cardiac biomarkers are the best predictors of survival outside of the transplantation setting. We now test whether cardiac troponins (cTnT and cTnI) and N-terminal pro-brain natriuretic peptide (NT-proBNP) are prognostic in transplant recipients. In 98 patients with AL undergoing PBSCT, serum cardiac biomarkers were measured (cTnT, 98 patients; cTnI, 65 patients; and NT-proBNP, 63 patients). Elevated levels of cTnT, cTnI, and NT-proBNP were present in 14%, 43%, and 48% of patients, respectively. At 20 months median follow-up, median survival has not been reached for patients with values below the thresholds; in patients with values above the thresholds, median survival is 26.1 months, 66.1 months, and 66.1 months, respectively. Our previously reported risk systems incorporating these markers were also prognostic, notably the cTnT/NT-proBNP staging. Using this system, 49%, 38%, and 13% of patients were in stage I, stage II, and stage III, respectively. Determining levels of circulating biomarkers may be the most powerful tool for staging patients with AL undergoing PBSCT.
原发性系统性淀粉样变性(AL)是一种致命的浆细胞疾病。初步数据表明,接受外周血干细胞移植(PBSCT)的患者生存率更高,但选择过程使这种明显的益处受到质疑。我们曾报道,循环心脏生物标志物是移植环境外生存的最佳预测指标。我们现在测试心肌肌钙蛋白(cTnT和cTnI)和N末端脑钠肽前体(NT-proBNP)在移植受者中是否具有预后价值。在98例接受PBSCT的AL患者中,检测了血清心脏生物标志物(cTnT,98例;cTnI,65例;NT-proBNP,63例)。cTnT、cTnI和NT-proBNP水平升高的患者分别占14%、43%和48%。在中位随访20个月时,低于阈值的患者尚未达到中位生存期;高于阈值的患者中位生存期分别为26.1个月、66.1个月和66.1个月。我们之前报道的纳入这些标志物的风险系统也具有预后价值,特别是cTnT/NT-proBNP分期。使用该系统,分别有49%、38%和13%的患者处于I期、II期和III期。测定循环生物标志物水平可能是对接受PBSCT的AL患者进行分期的最有力工具。