抄録
A case of systemic amyloidosis which was initially diagnosed by congo red staining on bone marrow aspirate is presented.
A 71-year-old male patient had been hospitalized since February 1977 under the diagnosis of nephrotic syndrome, and he was found to have monoclonal protein in the serum. To evaluate further his disease, he was admitted to our hospital in May 1977.
On physical examination, edema was noted at lower extremities without lymphadenopathy nor hepatosplenomegaly. Skeletal x-rays showed no abnormalities. An ECG demonstrated left axis deviation, clockwise rotation and diminished R waves in chest leads, suggesting amyloid heart. Blood urea nitrogen was 20 mg/dl, cholesterol 297 mg/dl and erythrocyte sedimentation rate 72 mm in one hour. The serologic test for syphilis was positive in the blood and in the spinal fluid. Total serum protein was 5.6 g/dl, and protein electrophoresis showed M peak in the fast gamma region. Urinalysis disclosed a 3+ test for protein. Immunoelectrophoretic analysis of the serum and urinary proteins identified an abnormal gamma peak as Ig A (λ) in the serum without Bence Jones protein in the urine. Peripheral blood showed moderate anemia and no atypical cells. An aspirate of sternal marrow was normocellular, revealed 22.4 per cent plasma cells including primitive form. It was also noted that an “amyloid” deposit was localized to the medullary vessels by histological studies of the clot section of the aspirate. Subsequently rectal and skin biopsies were positive for “amyloid” and a diagnosis of systemic amyloidosis was made.
