Castleman 씨 병에 동반된 신유전분증 = Renal Amyloidosis Associated with Castleman`s Disease

다국어 초록 (Multilingual Abstract)

Castlemam's disease is an asymptomatic and benign lymph node hyperplasia which is frequently manifested as mediastinal mass in over 70% of cases. Amyloidosis is rarely associated with Castleman's disease. Recently we experienced a patient with nephrotic syndrome due to renal amyloidosis who had been previously diagnosed as Castleman's disease of retroperitoneal lymph node enlargement. A 47-year old woman was admitted because of generalized edema and chest tightness. Five months ago she diagnosed as Castlman's disease-plasma cell type, of retroperitoneal lymph node enlargement. The patient was followed up through outpatient department without any specific medication. Physical examination showed pale conjunctivae, palpable mass on epigastric area and pretibial pitting edema. The hemoglobin, leukocyte count and erythrocyte sedimentation rate were 96 g/L, 5.9×109/L and 127 mm/hour, respectively. The blood urea nitogen was 3.6 mmol/L and creatinine was 88 umol/L. The urinalysis showed 3 positive for albumin. Twentyfour hour urine contained 16.6 grams of protein. A computerized tomographic (CT) scan of the abdomen disclosed multiple lymph node enlargement at mesentery. Percutaneous needle biopsy of kidney showed massive infiltration of pale pinkish amorphous material in the glomerular tuft forming large nodules. Ultrastructural examination exhibited heavy deposition of amyloid fibrils in the mesangium and perpendicular to glomerular basement membrane. Polarizing microscopy with Congo-red stain revealed prominent yellow green birefringence in glomeruli, tubules and interstitium.