Erythroleukemia (di Guglielmo Syndrome)

Discussion of a curious disease of the blood was begun by Copelli1in 1912 and continued by di Guglielmo.2,3Dameshek and Gunz5called 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