Tambuzzi Stefano, Andreola Salvatore, Boracchi Michele, Fociani Paolo, Gentile Guendalina, Zoja Riccardo
Dipartimento di Scienze Biomediche per la Salute, Sezione di Medicina Legale e delle Assicurazioni, Università degli Studi di Milano, Via Luigi Mangiagalli, 37, 20133, Milano, Italy.
Direttore UOC di Anatomia patologica, Via Papa Giovanni Paolo II, 20025, Legnano, Milano, Italy.
J Forensic Leg Med. 2020 Apr;71:101939. doi: 10.1016/j.jflm.2020.101939. Epub 2020 Mar 10.
In a previous study, we presented a case of an elderly woman's sudden death, in which microscopic examinations showed intramyocardial eosinophilic material suspected for amyloid, but not definable as such to the classic Congo Red staining. To overcome the arisen interpretative and diagnostic difficulties, we experimentally modified the classic Congo Red staining, using a specific one for corpse. The finding of a low-intensity positivity allowed us to formulate a very likely diagnosis of occult lethal cardiac amyloidosis. However, this low-intensity positivity obtained after having applied this experimental method for the first time and in only one case, as well as the existence of the rare pathology known as microfibrillar cardiomyopathy, which may be related to the observed microscopic findings, have forced us to investigate the correctness of the diagnosis. For this purpose, we performed in-depth investigations with sodium sulphate-Alcian Blue (SAB) staining and immunohistochemistry. Thanks to them, the amyloid nature of the intramyocardial material was confirmed and has been proved not only the reliability of our experimentally modified technique, but also the appropriateness of the diagnosis previously formulated. Therefore, the supposed involvement of the microfibrillar cardiomyopathy was excluded.
在之前的一项研究中,我们报告了一例老年女性猝死病例,其中显微镜检查显示心肌内有疑似淀粉样蛋白的嗜酸性物质,但经典刚果红染色无法明确其性质。为克服出现的解释和诊断困难,我们对经典刚果红染色进行了实验性改良,采用了一种针对尸体的特殊染色方法。低强度阳性结果使我们很可能诊断为隐匿性致死性心脏淀粉样变性。然而,首次应用这种实验方法且仅在一个病例中获得的这种低强度阳性结果,以及存在可能与观察到的显微镜下表现相关的罕见病理微纤维性心肌病,促使我们研究该诊断的正确性。为此,我们用硫酸铝-阿尔辛蓝(SAB)染色和免疫组织化学进行了深入研究。借助这些方法,心肌内物质的淀粉样蛋白性质得到证实,不仅证明了我们实验改良技术的可靠性,还证明了先前诊断的合理性。因此,排除了微纤维性心肌病的推测性累及。