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June 1957

Benign Purpura Hyperglobulinemica: Report of a Case

Author Affiliations


From the Departments of Hematologic Research, Medical Research Institute, and of Internal Medicine, Michael Reese Hospital. The Department of Hematologic Research is in part supported by the Michael Reese Research Foundation, and the Hulda B. and Maurice L. Rothschild Foundation for Scientific Research. Ruth Berger Reader Fellow, Hematology Research Foundation, 1955/1956 (Dr. Rozengvaig).

AMA Arch Intern Med. 1957;99(6):913-916. doi:10.1001/archinte.1957.00260060071007

In 1943, Waldenström, reviewing a large number of cases with hyperglobulinemia found three patients with a characteristic syndrome which he designated as "purpura hyperglobulinemica."1-3

This syndrome, seen predominantly in females after puberty, manifests itself by crops of petechiae or a purpuric rash, usually on the lower extremities, appearing at irregular intervals and occasionally preceded by a sensation of tenderness or even definite swelling of the involved limbs. The onset of the purpura has been related to overexertion or infections in a few cases. It usually disappears after a few days, and areas of brownish pigmentation may remain.

In the majority of cases, physical examination is noncontributory, although parotitis, conjunctivities, moderate generalized lymphadenopathy, hepatosplenomegaly, and tenderness of the lower extremities may be irregularly present.

Most of these patients have a normochromic normocytic anemia and an elevated sedimentation rate. Routine coagulation tests are normal, although Wilson et al.4 reported two