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March 1963

Erythroleukemia (di Guglielmo Syndrome): A Report of Clinical Observations and Experimental Studies in Seven Patients

Author Affiliations


Mead Johnson Postgraduate Scholar of American College of Physicians, 1958-1959, Senior Resident, Department of Medicine (Dr. Drevets); Professor, Department of Medicine (Drs. Sheets and Hamilton).; From the Department of Medicine, College of Medicine, University Hospitals, State University of Iowa.

Arch Intern Med. 1963;111(3):295-306. doi:10.1001/archinte.1963.03620270021004

Discussion of a curious disease of the blood was begun by Copelli1 in 1912 and continued by di Guglielmo.2,3 Dameshek and Gunz5 called it the "di Guglielmo syndrome" and defined it as a "myeloproliferative disorder." According to their concept, alterations in the bone marrow in erythroleukemia may pass through 3 phases if the patient lives long enough. The first phase is characterized by a preponderant erythroblastic proliferation with relatively minimal myeloblastic proliferation which is similar in description to di Guglielmo's3 "erythremic myelosis." The second stage is reached when the myeloblasts increase, and the term "erythroleukemia" becomes more appropriate. Presumably most patients come under observation during this phase. In the third and usually terminal phase, the "myeloblasts" become dominant and the bone marrow picture is similar to the blast crisis of acute or chronic myelocytic leukemia. Few published reports give data on these phases, and many are