高グロブリン血症性紫斑病(Waldenström)を伴つた橋本病の1例

抄録

A 52-year-old Japanese female was admitted to our hospital 15 years ago (Sept., 1956) with edema of the face and legs and shortness of breath. Her past and family histories were not remarkable. There were hyperproteinemia, rapid sedimentation rate and severe anemia (Table 1∼3).
Petechiae first developed over her both legs after prolonged laundry. In 1958 she was demonstrated to have marked increase in serum γ-globulin and in 6.7S component, abnormal decrease in BMR, PBI and ¹³¹I-uptake, and elevated serum total cholesterol; Bence Jones protein, pyroglobulin, macroglobul in and cryoglobulin were not demonstrable; the thyroid gland was slightly palpable but not enlarged. These findings led to a diagnosis of purpura hyperglobulinemica of Waldenström associated with idiopatic myxedema. The administration of thyroid preparation was followed by disappearance of the edema and improvement of anemia but had no effect on the serum protein pattern and purpura.
Close coagulation surveys were performed in 1964 (Table 4). The only finding of particular note was an unexplained poor response of patient's plasma to tissue thromboplastin, which was corrected by the addition of normal plasma.
In 1970, thyroid auto-antibodies were detected in the patient's serum; an open biopsy of the thyroid gland revealed histological findings of Hashimoto's disease (Fig. 4). Electrophoretic pattern showed a polyclonal increase in γ-globulin, and immunoelectrophoresis and immunodiffusion revealed also an simultaneous increase in IgG, IgA and IgM (Table 2, Fig. 3).
Recent coagulation studies demonstrated that the patient's plasma in the presence of thrombin at a low concentration gave a definitely prolonged thrombin time that was corrected by the addition of normal plasma, further addition of CaCl₂ or protamine sulfate (Table 5); these hemostatic abnormalities appear to be related to the purpura. An increase in antithrombin was entirely absent in this case.
The clinical picture and laboratory data hitherto obtained in the present case, except for the purpura, were identical with those of Hashimoto's disease known as one of the most representative autoimmune diseases. Waldenström proposed that purpura hyperglobulinemica might be an “auto-immune” condition. Our present case suggests that the syndrome is due to abnormal globulins produced by Hashimoto's disease.