Smith T J, Kyle R A, Lie J T
Mayo Clin Proc. 1984 Aug;59(8):547-55. doi: 10.1016/s0025-6196(12)61493-1.
Cardiac amyloidosis may be asymptomatic or an important cause of progressive heart failure and refractory arrhythmia. To identify the morphologic markers of clinically significant cardiac amyloidosis, we analyzed the hearts of 47 patients with autopsy-proven cardiac amyloidosis (21 with primary amyloidosis [AL] and 26 with senile cardiac amyloidosis [SCA]) histologically for the extent and pattern of amyloid deposits. The extent of amyloid deposition was graded 1 through 4, corresponding with less than 10%, 10 to 25%, 26 to 50%, and more than 50% histologic involvement of the myocardium, respectively. The pattern of deposits was classified as nodular, perifiber, or mixed type, and the presence or absence of vascular involvement was determined. The hearts with primary amyloidosis showed predominantly high-grade deposits (76% grades 3 and 4), a perifiber (65%) or mixed (30%) pattern of deposits, and frequent (90%) vascular involvement. The hearts with senile cardiac amyloidosis tended to have low-grade deposits (62% grades 1 and 2), a nodular pattern (92%) of deposits, and infrequent (4%) vascular involvement. Clinically significant cardiac amyloidosis was associated with grade 2 or greater amyloid deposits in the heart and with involvement of intramyocardial arterioles.
心脏淀粉样变性可能无症状，也可能是进行性心力衰竭和难治性心律失常的重要原因。为了确定具有临床意义的心脏淀粉样变性的形态学标志物，我们对47例经尸检证实为心脏淀粉样变性的患者（21例原发性淀粉样变性[AL]和26例老年心脏淀粉样变性[SCA]）的心脏进行了组织学分析，以了解淀粉样蛋白沉积的范围和模式。淀粉样蛋白沉积的程度分为1至4级，分别对应心肌组织学累及小于10%、10%至25%、26%至50%和大于50%。沉积物的模式分为结节状、纤维周围型或混合型，并确定是否存在血管受累。原发性淀粉样变性的心脏主要表现为高级别沉积（76%为3级和4级）、纤维周围（65%）或混合（30%）沉积模式，以及频繁（90%）的血管受累。老年心脏淀粉样变性的心脏倾向于低级别沉积（62%为1级和2级）、结节状沉积模式（92%）和罕见（4%）的血管受累。具有临床意义的心脏淀粉样变性与心脏中2级或更高等级的淀粉样蛋白沉积以及心肌内小动脉受累有关。
