抄録
Purpura hyperglobulinemica is a syndrome of which three cases featured with hyperglobulinemia and purpura were described by Waldenström for the first time in 1943, and in view of the subsequently reported cases, it is considered to comprise the primary and the secondary cases. In recent years, such secondary cases which accompany auto-immune diseases are often taken up.
Two cases of secondary purpura hyperglobulinemica have been described. Case 1 appeared secondary to myxedema and Sjögren syndrome. The antinuclear antibodies were positive. It was inferred that not only the elevation of γ-globulin level but also the decrease in platelet count, a reduction in platelet adhesiveness and aggregation, the presence of circulating anticoagulant, a tendency for euglobulin clot lysis time to be reduced, and abnormality in the correction of thrombin time were influential over the development of purpura in this case. The administration of thyroid hormone and corticosteroid resulted in the improvement in these symptoms. Case 2 appeared secondary to Sjögren syndrome and renal tubular acidosis. Not only γ-globulin level was elevated but also euglobulin clot lysis time tended to be reduced.
Because there are the “primary” and the “secondary” cases of purpura hyperglobulinemica, it has been proposed to indicate the identity as a remark to the diagnosis of this disease.