A 31-year-old male, taxi-driver, was first admitted to the Tokyo University Hospital on July 14, 1966, with the chief complaints of fever, general malaise, visual disturbance and general lymph node swelling. The swelling of the mesenteric lymph nodes had been noted in April 1964 on the appendectomy.
Physical examinations on admission revealed anemia, general lymphadenopathy and hepatosplenomegaly. Hemoglobin was 5.9 g/100 ml, RBC 1,910,000 with marked rouleaux formation, WBC 3,600 and platelets 130,000. A sternal marrow aspirate was hypercellular with 6.6 per cent early plasma cells. Urinalysis showed Bence Jones proteinuria. Total serum protein level was 12.0 g/100 ml with 79.7% of β-γ globulin. ESR 168 mm/hr (Westergren) and zinc turbidity test was over 40. Biopsy of the inguinal lymph node showed a plasma cell proliferation. Prednisolone and blood transfusions were began on the suspicion of multiple myeloma and the symptoms subsided. He was discharged on September 16, 1966. General malaise, fever and visual disturbance reappeared and he was readmitted on May 4, 1967.
On the second admission conditions were almost the same as were on the first. Immuno-electrophoresis of the patient's serum revealed the simultaneous increase in γG, γM and γA without M-component. Immunoelectrophoresis and Ouchterlony diffusion technic showed κ, λ, Fc and γG itself in the unconcentrated urine. Bone x-rays revealed no change.
Corticosteroid treatment and blood transfusions were again successful in controlling the complaints. Biopsy findings of the inguinal lymph node were almost the same as on the first admission. On July 19, he was diagnosed as the rupture of the right kidney, with sudden colicky pain and shock-like appearance. An emergency operation, right nephrectomy, was done. Histology of the resected kidney revealed the proliferation of the glomerular mesangium, the thickening of the basement membrane and marked, focal round cell infiltration in the interstitium. The cause of the kidney rupture remains unknown.
Simultaneous increase in γG, γM and γA was noted in the patient's serum and both heavy and light chains were demonstrated in his urine. The diagnosis shoud be, at present, “immunoglobulin fragment disease (Miyoshi)”.
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