Chopra S, Rubinow A, Koff R S, Cohen A S
Am J Pathol. 1984 May;115(2):186-93.
The liver is a major site of amyloid deposition. The spectrum of histopathologic changes in the liver was studied in 38 patients with systemic amyloidosis (25 with primary or myeloma-associated amyloidosis [AL] and 13 with secondary, reactive [AA] amyloidosis). Overall architectural distortion, alterations of portal triads, as well as predilection for topographic deposition in the parenchyma and/or blood vessel walls were noted. Significant histopathologic differences in AL or AA amyloid liver involvement included 1) portal fibrosis, seen in 7 of 25 (28%) AL patients and 8 of 13 (62%) AA patients (P = 0.05), 2) parenchymal amyloid deposition in 25 of 25 (100%) AL amyloid and 10 of 13 (77%) AA amyloid patients (P = 0.04), and 3) vascular amyloid deposition found in 17 of 25 (68%) with AL amyloid and 13 of 13 (100%) patients with AA amyloid (P = 0.02). These data vary from the widely held concept that deposition of amyloid is predominantly vascular in the AL form and parenchymal in amyloid AA. Clearly, however, in individual cases significant overlap occurred, and characterization of amyloid types based on morphologic distribution of amyloid deposits may be possible in only a minority of cases. In most cases, differentiation of amyloid AL and amyloid AA forms requires clinical, histochemical, immunochemical, and sometimes more elaborate laboratory amino acid sequence studies for accurate identification.
肝脏是淀粉样蛋白沉积的主要部位。我们对38例系统性淀粉样变性患者（25例原发性或骨髓瘤相关性淀粉样变性[AL]和13例继发性反应性[AA]淀粉样变性）肝脏的组织病理学变化谱进行了研究。观察到整体结构扭曲、门三联体改变，以及实质和/或血管壁内特征性的沉积倾向。AL或AA淀粉样变性肝脏受累的显著组织病理学差异包括：1）门脉纤维化，25例AL患者中有7例（28%）出现，13例AA患者中有8例（62%）出现（P = 0.05）；2）实质淀粉样蛋白沉积，25例AL淀粉样变性患者全部出现（100%），13例AA淀粉样变性患者中有10例出现（77%）（P = 0.04）；3）血管淀粉样蛋白沉积，25例AL淀粉样变性患者中有17例出现（68%），13例AA淀粉样变性患者全部出现（100%）（P = 0.02）。这些数据与普遍认为的淀粉样蛋白沉积在AL型中主要为血管性、在AA型中主要为实质性的概念不同。然而，显然在个别病例中存在显著重叠，仅在少数病例中基于淀粉样蛋白沉积物的形态学分布来鉴定淀粉样蛋白类型才是可能的。在大多数情况下，则需要临床、组织化学、免疫化学，有时还需要更精细的实验室氨基酸序列研究才能准确鉴别AL淀粉样蛋白和AA淀粉样蛋白。
