Gertz Morie A, Comenzo Ray, Falk Rodney H, Fermand Jean Paul, Hazenberg Bouke P, Hawkins Philip N, Merlini Giampaolo, Moreau Philippe, Ronco Pierre, Sanchorawala Vaishali, Sezer Orhan, Solomon Alan, Grateau Giles
Dysproteinemia Clinic, Mayo Clinic, Rochester, Minnesota 55905, USA.
Am J Hematol. 2005 Aug;79(4):319-28. doi: 10.1002/ajh.20381.
We undertook this study to develop uniformly accepted criteria for the definition of organ involvement and response for patients on treatment protocols for immunoglobulin light-chain amyloidosis (AL). A consensus panel was convened comprising 13 specialists actively involved in the treatment of patients with amyloidosis. Institutional criteria were submitted from each, and a consensus was developed defining each organ involved and the criteria for response. Specific criteria have been developed with agreed on definitions of organ and hematologic response as a result of discussions at the 10th International Symposium on Amyloid and Amyloidosis held in Tours, France, April 2004. These criteria now form the working definition of involvement and response for the purposes of future data collection and reporting. We report criteria that centers can now use to define organ involvement and uniform response criteria for reporting outcomes in patients with light-chain AL.
我们开展这项研究,旨在为免疫球蛋白轻链淀粉样变性(AL)患者治疗方案中器官受累及反应的定义制定统一认可的标准。召集了一个由13位积极参与淀粉样变性患者治疗的专家组成的共识小组。每位专家都提交了各自机构的标准,并形成了一份共识,明确了每个受累器官及反应标准。经过2004年4月在法国图尔举行的第10届国际淀粉样变性和淀粉样变研讨会上的讨论,已制定出具体标准,对器官和血液学反应的定义达成了一致。这些标准现构成了未来数据收集和报告目的下受累及反应的工作定义。我们报告了各中心现在可用于定义器官受累的标准以及用于报告轻链AL患者治疗结果的统一反应标准。