Hachulla E, Maulin L, Deveaux M, Facon T, Blétry O, Vanhille P, Wechsler B, Godeau P, Levesque H, Hatron P Y, Huglo D, Devulder B, Marchandise X
Department of Internal Medicine, Hôpital Claude Huriez, Centre Hospitalier Régional, University of Lille, France.
Am J Med. 1996 Jul;101(1):77-87. doi: 10.1016/s0002-9343(94)00054-9.
The purpose of this study was to assess the value of the serum amyloid P (SAP) component scintigraphy in patients with primary amyloidosis (AL).
Pure human SAP labeled with iodine-123 (123I-SAP) was given intravenously to 24 patients with biopsy-proven systemic amyloidosis (15 without multiple myeloma = group 1, and 9 with multiple myeloma = group 2) and to 6 patients with multiple myeloma without any clinical or biological signs of amyloidosis (group 3). Whole-body images as well as regional views and tissue retention levels were obtained after 24 hours. Our study was approved by the institutional review committee and all individuals gave informed consent and were prospectively studied (median 13 months, range 1 to 47 from the date of the scintigraphy to May 1995).
Organ localization of 123I-SAP, indicating the presence of substantial visceral amyloid deposits, was observed in all patients in group 1 and 2. The organ uptake of 123I-SAP included the spleen (1 patient was splenectomized) in 20 of 23 cases (87%), the liver in 15 of 24 (60%), and the kidneys in 6 of 24 (25%). Myocardial 123I-SAP was never seen although 13 out of the 24 patients had clinical or echographic data for amyloidosis. Twenty-four hour tissue retention was significantly elevated in all patients (group 1 and group 2): 55.66% +/- 19.16% in group 1 and 34.37% +/- 24.92% in group 2, as compared with normal levels < 24%. The sensitivity of the technique was 79% when only organ uptake was considered but reached 100% when tissue retention was also considered. The 24-hour tissue retention might be correlated with the severity of the amyloidosis: mean survival in patients with tissue retention greater than 50% was 11.3 months versus 24.5 months in patients with levels less or equal to 50%. Five of the 6 patients with multiple myeloma without evidence of amyloidosis had abnormal 123I-SAP imaging and 24-hour tissue retention levels. In 2 of them, amyloidosis was secondly detected. In the 9 patients who had two scintigraphies, variations in 24-hour tissue retention values were in accordance with the clinical evaluation.
Spleen and liver distribution of amyloidosis is mostly revealed by 123I-SAP scintigraphy in patients with AL amyloidosis. The uptake of 123I-SAP appeared in proportion to the quantity of amyloidosis present in different tissues, and the relative quantity of amyloid deposits in the myocardium, carpal tunnel, digestive tract, and kidneys was often small and seldom visualized by 123I-SAP scintigraphy. In contrast 24-hour tissue retention levels were abnormal in all cases of known AL amyloidosis. This may be a positive argument for the diagnosis of amyloidosis when histopathological tests are normal. Tissue retention levels appear important as they may be correlated with survival.
本研究旨在评估血清淀粉样蛋白P（SAP）成分闪烁扫描术在原发性淀粉样变性（AL）患者中的价值。
将用碘 - 123（123I - SAP）标记的纯人SAP静脉注射给24例经活检证实为系统性淀粉样变性的患者（15例无多发性骨髓瘤 = 第1组，9例有多发性骨髓瘤 = 第2组）以及6例无任何淀粉样变性临床或生物学迹象的多发性骨髓瘤患者（第3组）。24小时后获取全身图像以及局部视图和组织滞留水平。本研究经机构审查委员会批准，所有个体均签署知情同意书并进行前瞻性研究（从闪烁扫描术日期至1995年5月的中位数为13个月，范围为1至47个月）。
在第1组和第2组的所有患者中均观察到123I - SAP的器官定位，表明存在大量内脏淀粉样蛋白沉积。23例中有20例（87%）的123I - SAP器官摄取包括脾脏（1例已行脾切除术），24例中有15例（60%）包括肝脏，24例中有6例（25%）包括肾脏。尽管24例患者中有13例有淀粉样变性的临床或超声心动图数据，但从未观察到心肌摄取123I - SAP。所有患者（第1组和第2组）的24小时组织滞留均显著升高：第1组为55.66%±19.1%，第2组为34.37%±24.92%，而正常水平<24%。仅考虑器官摄取时该技术的敏感性为79%，但同时考虑组织滞留时则达到100%。24小时组织滞留可能与淀粉样变性的严重程度相关：组织滞留大于50%的患者平均生存期为11.3个月，而水平小于或等于50%的患者为24.5个月。6例无淀粉样变性证据的多发性骨髓瘤患者中有5例123I - SAP成像和24小时组织滞留水平异常。其中2例随后检测出淀粉样变性。在9例进行了两次闪烁扫描的患者中，24小时组织滞留值的变化与临床评估一致。
123I - SAP闪烁扫描术在AL淀粉样变性患者中大多能显示淀粉样变性在脾脏和肝脏的分布。123I - SAP的摄取与不同组织中存在的淀粉样变性数量成比例，而心肌、腕管、消化道和肾脏中淀粉样蛋白沉积的相对数量通常较少，很少能通过123I - SAP闪烁扫描术显示。相比之下，所有已知的AL淀粉样变性病例中24小时组织滞留水平均异常。当组织病理学检查正常时，这可能是诊断淀粉样变性的一个有力依据。组织滞留水平似乎很重要，因为它们可能与生存期相关。
