Güz G, Ozdemir B H, Sezer S, Yakupoglu U, Demirhan B, Ozdemir F N, Haberal M
Department of Nephrology, Baskent University Faculty of Medicine, Ankara, Turkey.
Ren Fail. 2000;22(5):613-21. doi: 10.1081/jdi-100100902.
Amyloid lymphadenopathy has only been reported in case report form, or in small groups of patient groups within large series. We believe that amyloid lymphadenopathy is common in uremic patients, and thus designed this study to determine the frequency of this condition in hemodialysis patients, and to assess its types and patterns. We reevaluated 46 uremic patients' lymph node biopsies for amyloid deposits. We also immunohistochemically identified the protein origin of these deposits using Amyloid A, kappa, lambda, beta2 microglobulin, and transthyretin antibodies. Histopathologically, we observed for vascular involvement, follicular deposition, and diffuse deposition. We detected amyloid deposits in 10 of the 46 (22%) patients' lymph nodes. The patterns of deposition were vascular involvement alone in six specimens, vascular involvement plus follicular deposition in three, and vascular involvement plus diffuse deposition in one specimen. Amyloid AA type protein was present in seven nodes, beta2 microglobulin-related amyloid in two nodes, and immunoglobulin-derived protein (AL) in one node. We assessed these 10 patients for causes of end-stage renal disease (ESRD) and other conditions that might relate to amyloidosis. The cause of ESRD in the seven patients with AA amyloid were renal amyloidosis secondary to Familial Mediterranean Fever in four, glomerulonephritis in one patient who had bronchiectasis and Castleman's disease, unknown in one patient who had bronchial asthma, and pyelonephritis in one patient who had no characteristics that could be linked with AA type amyloidosis. The causes of ESRD in the two individuals with beta2 microglobulin-related amyloidosis who had been on long-term hemodialysis were pyelonephritis and glomerulonephritis. The cause of ESRD in the patient with AL type protein was glomerulonephritis, and this patient had no systemic disease. We conclude that amyloid lymphadenopathy is, indeed, common in uremic patients. Amyloid type AA is the most prevalent form of amyloid protein in uremic patients, but amyloid type does not always correspond with underlying cause of renal failure, or with the presence of systemic disease.
淀粉样变淋巴结病仅以病例报告形式报道过,或在大型系列研究中的小部分患者群体中有所提及。我们认为淀粉样变淋巴结病在尿毒症患者中很常见,因此设计了本研究以确定血液透析患者中这种疾病的发生率,并评估其类型和模式。我们重新评估了46例尿毒症患者的淋巴结活检组织以寻找淀粉样沉积物。我们还使用淀粉样蛋白A、κ、λ、β2微球蛋白和转甲状腺素蛋白抗体通过免疫组化鉴定了这些沉积物的蛋白质来源。在组织病理学方面,我们观察了血管受累情况、滤泡沉积和弥漫性沉积。我们在46例患者中的10例(22%)淋巴结中检测到了淀粉样沉积物。沉积模式为6个标本仅有血管受累,3个标本血管受累加滤泡沉积,1个标本血管受累加弥漫性沉积。7个淋巴结中存在淀粉样蛋白AA型,2个淋巴结中存在β2微球蛋白相关淀粉样蛋白,1个淋巴结中存在免疫球蛋白衍生蛋白(AL)。我们评估了这10例患者的终末期肾病(ESRD)病因以及其他可能与淀粉样变性相关的情况。7例AA型淀粉样蛋白患者的ESRD病因中,4例继发于家族性地中海热的肾淀粉样变性,1例患有支气管扩张和Castleman病的患者为肾小球肾炎,1例患有支气管哮喘的患者病因不明,1例患有肾盂肾炎但无与AA型淀粉样变性相关特征。2例长期血液透析的β2微球蛋白相关淀粉样变性患者的ESRD病因是肾盂肾炎和肾小球肾炎。AL型蛋白患者的ESRD病因是肾小球肾炎,且该患者无全身性疾病。我们得出结论,淀粉样变淋巴结病在尿毒症患者中确实很常见。AA型淀粉样蛋白是尿毒症患者中最常见的淀粉样蛋白形式,但淀粉样蛋白类型并不总是与肾衰竭的潜在病因或全身性疾病的存在相对应。