87例肾淀粉样变的临床病理分析 word格式.docxVIP

Clinico-pathological Study of 87 patients of renal amyloidosisAbstractObjective: To analyze the clinico-pathological data of renal amyloidosis and to investigate the immunohistochemical typing and diagnosis of familial renal amyloidosis.Methods: 87 patients of amyloidosis admitted in department of nephrology of Shanghai Ruijin Hospital from April 1990 to March 2008 were enrolled in this retrospective clinico-pathological study. Clinico-pathological data of one pedigree of familial renal amyloidosis were collected. Data were analyzed by SPSS 13.0.Results: 50 patients were male, 37 were female and sex ratio was 1.35:1. The average age was 54.7±10.9 years. 83 patients had primary amyloidosis (AL type) including 14 patients associated with multiple myeloma (MM). 2 patients had secondary amyloidosis (AA type). 2 patients had familial renal amyloidosis. 3.4% of patients had hypertension while 8.0% had hypotension. 21.8% of patients had microscopic hematuria. The median quantity of 24-hour urinary protein was 4200mg. The average renal length was106.0±9.8mm. 26.4% of patients had thickness of interventricular septum more than 12mm. 8.0% of patients had hepatomegaly while 12.6% of patients had splenomegaly. 53 patients had test of serum immunoelectrophoresis and a monoclonal protein was detected in 54.7% of patients. 64 patients had bone marrow biopsy in which 75.0% of patients had AL amyloidosis without MM and median plasmocyte percentage was 2.5% while 21.9% of patients had AL amyloidosis associated with MM and median plasmocyte percentage was 16.5%. 47.0% of patients received chemotherapy of melphalan and prednisone (MP) while 53.0% of patients had Vincristin/Adriamycin/Dexamethasone (VAD).All renal tissues were positive for congo red and had apple green birefringence under polarized light. Amyloid protein was substantially localized in glomerular basement membrane, mesangial area and inner arteriolar membrane and it was partially localized in tubulo-interstitial