Abstract
The patient is a 61-years-old woman. Her family history and past history was nonsontributory. She was admitted to our hospital (from April to May, 1979) for the first time because of facial edema. The laboratory findings showed the following results : Uri-nary protein (+++), B-J protein (-), IgG (λ) M-protein in the serum 1.16 g/dl, and plasmacell count in the bone marrow 5.60. The facial edema was relieved by the administration of a diuretic agent. She was discharged and followed up as an outpatient. Since January 1981, however, nephrotic syndrome occurred, and B-J protein and increased serum Mprotein were observed. In addition, anemia occurred, and she was admitted again (from June 1981 to January 1982). She had macroglossia, hepatomegaly, splenomegaly, and hoarseness, but swelling of the lymph nodes was not found. Immediately after the admission, the biopsy of the labial gland was performed. The result indicated that a substance which could be specifically stained with H-E and Congo Red had deposited in the minor salivary gland. A diagnosis of amyloidosis was made. Furthermore, an open right kidney biopsy disclosed marked deposition of a substance which reacted positive with the same stain on the vascular walls of the glomerulus. DMSO, 40 mg/Kg was administered orally to the patient for primary amyloidosis parallel to the treatment of nephrotic syndrome.
However, IgG (λ) M-protein in the serum was unchanged, B-J (λ) M-protein in urine was elevated, and anemia and renal function were increasingly aggravated. In October 1981 plasma cell count in the bone marrow was 29% and the cells were atypical, and concomitant MP therapy was started for multiple myeloma. However, systemic edema incr-eased and she died on January 22, 1982 Major autopsy findings revealed the following findings. Edema was marked throughout the whole body including 1000 ml of bilateral pleural fluid and 3000 ml of light yellow cloudy ascites. Small foci of invasion were scattered here and there in the sternum and vertebrae, which showed a picture of multiple myeloma histologically, but extramedullary infiltration of tumor cells were not detected. The deposits of amyloid substance were found on the vascular walls of nearly the whole body ; there were deposits in the intercellular substance and muscle fibers in the heart, and highly diffuse ones on the glomerulus in the kidney which were nealy hyalinized. Deposition occurred almost diffusely in the red pulp, and the follicles were rather atrophic in the spleen. In addition, deposits were found in the intracellular substance and muscle fibers of the tongue, in the intracellular substance of the digestive tract and on the dermis of the skin. The present case was a generalized amyloidosis associated with multiple myeloma. As secondary lesions, pulmonary edema, congestion of the lung, liver and spleen, and nonspecfic acute colitis were observed.
