肾淀粉样变的诊断与病理分型分析

Objective To clarify the clinicopathological features of renal amyloidosis in order to achieve early diagnosis and treatment. Methods Clinicopathological data of 26 biopsy-proven renal amyloidosis cases in Department of Nephrology, Zhongshan Hospital, Fudan University between 2006 and 2010 were analyzed retrospectively. Immunohistochemistry and immunofluorescence of amyloid A protein, immunoglobulin light chains such as ?资、?姿 were performed on renal specimens for further classification. Results Age of 26 patients ranged from 40 to 77 years old, average (58.54±10.07) years. Twenty-two out of 26 patients (84.62%) were treated in local hospital before admitted to our department, and 21 patients (95.45%) were misdiagnosed as chronic primary glomerulonephritis. The prominent clinical manifestations of renal amyloidosis were nephrotic syndrome (17 cases, 65.38%), decreased blood pressure (16 cases, 61.53%), organ enlargement (8 cases, 30.77%) and bodyweight loss (6 cases, 23.08%). Fourteen out of 25 patients (56.00%) were found to have monoclonal light chains in serum by immunofixation electrophoresis. Three patients with mild pathological changes who had no confirmable Congo red stain were confirmed by electron microscopy. Twenty-three (88.46%) patients were diagnosed as AL amyloidosis, one (3.85%) as AA amyloidosis, one was strongly suspected of hereditary amyloidosis, and one was undetermined. Conclusions Renal amyloidosis is frequently misdiagnosed. Middle-aged and old nephrotic patients with decreased blood presure, organ enlargement and bodyweight loss may be the most helpful clues of the disease. Most patients have monoclonal light chains in serum or urine. Renal biopsy, especially electronic microscopy plays a crucial role in the early diagnosis of renal amyloidosis. Immunohistochemistry is important for patients with renal amyloidosis in pathological classification and treatment.

Clinicopathology, diagnosis and classification of renal amyloidosis

Objective To clarify the clinicopathological features of renal amyloidosis in order to achieve early diagnosis and treatment. Methods Clinicopathological data of 26 biopsyproven renal amyloidosis cases in Department of Nephrology,Zhongshan Hospital,Fudan University between 2006 and 2010 were analyzed retrospectively. Immunohistochemistry and immunofluorescence of amyloid A protein,immunoglobulin light chains such as K、λ were performed on renal specimens for further classification. Results Age of 26 patients ranged from 40 to 77 years old,average (58.54±10.07) years.Twenty-two out of 26 patients (84.62％) were treated in local hospital before admitted to our department,and 21 patients (95.45％) were misdiagnosed as chronic primary glomerulonephritis.The prominent clinical manifestations of renal amyloidosis were nephrotic syndrome (17 cases,65.38％),decreased blood pressure (16 cases,61.53％),organ enlargement (8 cases,30.77％) and bodyweight loss (6 cases,23.08％).Fourteen out of 25 patients (56.00％) were found to have monoclonal light chains in serum by immunofixation electrophoresis.Three patients with mild pathological changes who had no confirmable Congo red stain were conffimed by electron microscopy. Twenty-three (88.46％) patients were diagnosed as AL amyloidosis,one (3.85％) as AA amyloidosis,one was strongly suspected of hereditary amyloidosis,and one was undetermined. Conclusions Renal amyloidosis is frequently misdiagnosed.Middleaged and old nephrotic patients with decreased blood presure,organ enlargement and bodyweight loss may be the most helpful clues of the disease.Most patients have monoclonal light chains in serum or urine.Renal biopsy,especially electronic microscopy plays a crucial role in the early diagnosis of renal amyloidosis.Immunohistochemistry is important for patients with renal amyloidosis in pathological classification and treatment.