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November 1957

Purpura of Idiopathic HyperglobulinemiaWaldenström's Purpura Hyperglobulinemica

AMA Arch Derm. 1957;76(5):575-583. doi:10.1001/archderm.1957.01550230041007

Hemorrhagic disturbances may be classified in four groups on the basis of their presumptive pathogenetic mechanism as follows: (1) those due to a primary defect of the vascular wall; (2) those due to a qualitative or quantitative platelet deficiency; (3) those due to abnormalities in the blood coagulation factors, and (4) those due to qualitative or quantitative disturbances of the blood proteins (dysproteinemias).19

Related to the last group, Waldenström16 described a new syndrome, namely "purpura hyperglobulinemica," characterized by relapsing purpura of the lower extremities, persistent increase of the serum-globulin fraction, mild normochromic anemia, leukopenia, elevated erythrosedimentation rate (E. S. R.) and increased total serum proteins between 8.7 and 9.8 gm. per 100 cc. Waldenström performed electrophoretic studies on the three original patients and four additional cases.17,18 These studies disclosed the following: The hyperglobulinemia was due to an increase of

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