Abstract：
OBJECTIVE: To determine the prevalence of different amyloid types and frequency of associated systemic amyloidosis in the urinary tract/prostate.
METHODS: We studied Congo red-positive prostate (n = 150) and urinary tract (n = 767) specimens typed by a proteomics-based method between 2008 and 2020. Clinical follow up was available for a subset (urinary tract, n = 111; prostate, n = 17). Amyloid types were correlated with various clinicopathologic features. For patients with clinical follow up, chart review was performed to establish localized versus systemic disease, frequency of initial diagnosis of amyloidosis on urinary tract/prostate specimens, presence of cardiac disease, and death from disease-related complications.
RESULTS: The most common amyloid types were AL/AH in urinary tract (479/767, 62 %) and localized ASem1 in prostate (64/150, 43 %). Urinary tract AL/AH amyloid was usually localized, but systemic AL amyloidosis occurred in both sites (urinary tract: 5/71, 7 %; prostate: 2/2, 100 %). ATTR amyloidosis was seen in over a third of cases (urinary tract: 286/767, 37 %; prostate: 55/150, 37 %). Urinary tract/prostate was the site of the initial ATTR amyloidosis diagnosis in 44/48 patients (92 %), and 38/48 (79 %) were subsequently found to have cardiac involvement. Seminal vesicle/ejaculatory duct involvement was pathognomonic for ASem1-type amyloidosis (39/39, 100 %).
CONCLUSIONS: Over 40 % of patients had systemic amyloidosis, with urinary tract/prostate often the first site in which amyloid was identified. Since early recognition of systemic amyloidosis is critical for optimal patient outcomes, there should be a low threshold to perform Congo red stain. Proteomics-based amyloid typing is recommended since treatment depends on correctly identifying the amyloid type.
METHODS: We studied Congo red-positive prostate (n = 150) and urinary tract (n = 767) specimens typed by a proteomics-based method between 2008 and 2020. Clinical follow up was available for a subset (urinary tract, n = 111; prostate, n = 17). Amyloid types were correlated with various clinicopathologic features. For patients with clinical follow up, chart review was performed to establish localized versus systemic disease, frequency of initial diagnosis of amyloidosis on urinary tract/prostate specimens, presence of cardiac disease, and death from disease-related complications.
RESULTS: The most common amyloid types were AL/AH in urinary tract (479/767, 62 %) and localized ASem1 in prostate (64/150, 43 %). Urinary tract AL/AH amyloid was usually localized, but systemic AL amyloidosis occurred in both sites (urinary tract: 5/71, 7 %; prostate: 2/2, 100 %). ATTR amyloidosis was seen in over a third of cases (urinary tract: 286/767, 37 %; prostate: 55/150, 37 %). Urinary tract/prostate was the site of the initial ATTR amyloidosis diagnosis in 44/48 patients (92 %), and 38/48 (79 %) were subsequently found to have cardiac involvement. Seminal vesicle/ejaculatory duct involvement was pathognomonic for ASem1-type amyloidosis (39/39, 100 %).
CONCLUSIONS: Over 40 % of patients had systemic amyloidosis, with urinary tract/prostate often the first site in which amyloid was identified. Since early recognition of systemic amyloidosis is critical for optimal patient outcomes, there should be a low threshold to perform Congo red stain. Proteomics-based amyloid typing is recommended since treatment depends on correctly identifying the amyloid type.
摘要：
目的：确定尿路/前列腺中不同淀粉样蛋白类型的患病率和相关系统性淀粉样变性的频率。
方法：我们在2008年至2020年期间研究了刚果红阳性前列腺（n=150）和尿路（n=767）标本，采用基于蛋白质组学的方法进行分型。临床随访可用于一个子集(尿路，n=111；前列腺，n=17）。淀粉样蛋白类型与各种临床病理特征相关。对患者进行临床随访,进行图表审查以建立局部疾病与全身性疾病，尿路/前列腺标本上淀粉样变性的初始诊断频率，心脏病的存在，和死于疾病相关并发症。
结果：最常见的淀粉样蛋白类型是尿路AL/AH（479/767，62％）和前列腺局部ASem1（64/150，43％）。尿路AL/AH淀粉样蛋白通常是局部的，但系统性AL淀粉样变性发生在两个部位(尿路:5/71,7%;前列腺:2/2,100%)。在超过三分之一的病例中观察到ATTR淀粉样变性(尿路:286/767,37%;前列腺:55/150,37%)。尿路/前列腺是44/48例患者（92%）中ATTR淀粉样变性的初始诊断部位，38/48（79％）随后发现心脏受累。精囊/射精管受累是ASem1型淀粉样变性的病因（39/39，100％）。
结论：超过40%的患者有系统性淀粉样变性，泌尿道/前列腺通常是发现淀粉样蛋白的第一个部位。由于早期识别系统性淀粉样变性对于最佳患者预后至关重要，刚果红染色应该有一个低门槛。建议使用基于蛋白质组学的淀粉样蛋白分型，因为治疗取决于正确识别淀粉样蛋白类型。
方法：我们在2008年至2020年期间研究了刚果红阳性前列腺（n=150）和尿路（n=767）标本，采用基于蛋白质组学的方法进行分型。临床随访可用于一个子集(尿路，n=111；前列腺，n=17）。淀粉样蛋白类型与各种临床病理特征相关。对患者进行临床随访,进行图表审查以建立局部疾病与全身性疾病，尿路/前列腺标本上淀粉样变性的初始诊断频率，心脏病的存在，和死于疾病相关并发症。
结果：最常见的淀粉样蛋白类型是尿路AL/AH（479/767，62％）和前列腺局部ASem1（64/150，43％）。尿路AL/AH淀粉样蛋白通常是局部的，但系统性AL淀粉样变性发生在两个部位(尿路:5/71,7%;前列腺:2/2,100%)。在超过三分之一的病例中观察到ATTR淀粉样变性(尿路:286/767,37%;前列腺:55/150,37%)。尿路/前列腺是44/48例患者（92%）中ATTR淀粉样变性的初始诊断部位，38/48（79％）随后发现心脏受累。精囊/射精管受累是ASem1型淀粉样变性的病因（39/39，100％）。
结论：超过40%的患者有系统性淀粉样变性，泌尿道/前列腺通常是发现淀粉样蛋白的第一个部位。由于早期识别系统性淀粉样变性对于最佳患者预后至关重要，刚果红染色应该有一个低门槛。建议使用基于蛋白质组学的淀粉样蛋白分型，因为治疗取决于正确识别淀粉样蛋白类型。
